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Peripheral Ulcerative Keratits

Peripheral ulcerative keratitis (PUK) is a destructive inflammatory disease of the peripheral cornea characterized by epithelial defects, stromal thinning and infiltration, and potential perforation. It can be caused by autoimmune, infectious, or inflammatory processes. Treatment involves topical lubricants, antibiotics, corticosteroids, and systemic immunosuppressants depending on severity and underlying etiology to stop corneal melting and promote healing. A thorough workup is needed to identify any associated systemic diseases.

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DR. RESHMA PETER PERIPHERAL ULCERATIVE KERATITIS
A group of Destructive inflammatory diseases involving the Peripheral cornea Final common pathway  sloughing of corneal epithelium and keratolysis (corneal melting) • Begins with crescentic destructive inflammation at corneal periphery • Ass w/ epithelial defects, stromal inflammatory cells, Progressive stromal melting , degradation and necrosis perforation • May be associated with Episcleritis, Necrotizing Scleritis, Iridocyclitis
PERIPHERAL CORNEA • No clear-cut borders delineating peripheral cornea • Portion located b/w central 50% of cornea and limbus. • An arbitrary central limit beginning at 3.5–4.5 mm from the visual axis extending to junction of ill-defined transition b/w limbus and conjunctiva. • Directly adjacent to limbus and internal angle structures • Highly vascular zone with associated lymphatic tissue, scleral collagen, corneal collagen and limbal stem cells. • Thus, vascular inflammatory disorders, limbal infections, collagen vascular disorders, neoplastic disease, and local degenerations may affect peripheral cornea in a distinctive way
Distinct anatomic differences of the peripheral cornea • Thickest region of the cornea (up to 0.7 mm) with tight collagen bundle packing • A vascular arcade originating from the anterior ciliary arteries extends approx. 0.5 mm into the clear cornea • Unlike central avascular cornea , peripheral cornea derives part of its blood supply from Ant conjunctiva and deep episcleral vessels which are a source of immunocompetent cells • The accompanying lymphatics, which drain to the regional lymph nodes.
 The adjacent conjunctival blood vessels and lymphatics provide the peripheral cornea • Access to afferent and efferent arc of the immune system • Source of inflammatory effector cells and cytokines involved in the production of collagenase and proteoglycanase, which contribute to corneal degradation.  Unique immunologic characteristics of the peripheral cornea • presence of more Langerhans’ cells, • higher concentrations of IgM and C1 (important in the activation of the classic complement pathway), due to diffusion from limbal vessels due to large size
ETIOLOGY  Majority- local and systemic immunological processes  Few cases - infectious Ocular Non-infectious Infectious • Idiopathic • Acne rosacea (ocular) • Mooren’s ulcer • Traumatic, post surgical • Exposure/neuroparalytic keratopathy • Marginal keratitis • Contact lens use • Nutritional deficiencies • Bacterial (Staphylococcus, Streptococcus, Gonococcus [rare] • Viral (HSV,HZV) • Amoebic (Acanthamoeba) • Fungal
Systemic Noninfectious Infectious Collagen vascular diseases/vasculitis • Rheumatoid arthritis • ANCA-associated vasculitides • Wegener’s granulomatosis • Polyarteritis nodosa • Microscopic polyangiitis • Churg–Strauss syndrome • Relapsing polychondritis • SLE • Progressive systemic sclerosis or scleroderma • Giant cell arteritis Other systemic autoimmune • Sjögren’s syndrome • Cicatricial pemphigoid (especially with trichiasis) • Inflammatory bowel disease • Sarcoidosis • Leukemia/malignancy • Giant cell arteritis • Gonorrhea • Bacillary dysentery- Salmonella, Shigella • Tuberculosis • Syphilis • Varicella zoster • Helminthiasis • Hep C • HIV • Borreliosis(Lyme disease )
PATHOGENESIS Exact pathophysiologic mechanisms - unclear. PROPOSED MECHANISMS IN IMMUNOLOGICAL PATHOGENESIS  Circulating immune complex deposition  Autoimmune reactions to corneal antigens  Hypersensitivity reactions to exogenous antigens Evidence suggests that both humoral and cell-mediated mechanisms (T cell and B cell) are involved.
Various Etiologies Inflammatory reaction at corneal periphery Immune complex deposition and Complement Activation (C1) Immune vasculitis (damage to the vessel wall ) Further increase in vascular permeability causing leakage Generates chemotactic factors for neutrophils,pro inflammatory cytokines (C3a, C5a) Neutrophils recruited at peripheral cornea liberate proteolytic and collagenolytic enzymes Metalloproteinases production by local resident cells Destruction of corneal stroma
INFECTIOUS PATHOGENESIS Microorganisms produce proteases  disrupt the extracellular matrix Local productions of cytokines and chemokines diapedesis and migration of neutrophils into peripheral cornea from limbal vessels Cornea inflammation Enzymes released by neutrophils and corneal matrix metalloproteinases activation exacerbate inflammatory necrosis corneal perforation Antimicrobial control of bacterial replication wound healing processes NV vs scarring
Superinfection • Sta. aureus -Adhesins bind to collagen and other components of exposed Bowman and stroma. • Pseudomonas aeruginosa can bind to molecular receptors exposed on injured epithelial cells. Signs of resolution of active PUK  Resolution of the conjunctival inflammation  Epithelialization of the corneal surface with residual corneal thinning and a vascularized scar.
OCULAR MANIFESTATIONS Presenting symptoms of PUK -not specific. Foreign body sensation, watering, pain, and photophobia - epithelial erosion and ulceration. Pain is severe - associated scleritis Pain without scleral involvement- Mooren’s Ulcer Decreased Vision - inflammatory process proceeds centrally. • Photophobia with reduced visual acuity - Associated anterior uveitis
Slit lamp Examination • Crescent shaped destructive lesion at juxta limbal corneal stroma • W/in 2mm of limbus ,accompanied by varying degrees of vaso-occlusion of adjacent limbal networks • Epithelial defect • Stromal thinning w/ yellow white infiltrates –inflammatory cells • In early ds., patchy epithelial involvement;stroma –near normal thickness • Varying degrees of corneal thinning Thinning due to keratolysis (corneal melting), may occur at any clock hour of the peripheral cornea, with or w/out an intervening clear zone from limbus
• Disease process may progress circumferentially, centrally, and posteriorly  perforation. • Corneal melting may progress very rapidly • Amount of stromal loss underappreciated due to debris and necrotic material deposited at the base of the ulcer. • Becomes more apparent after scrapings
• Frequently limbal, conjunctival, and episcleral injection occurs. • Concurrent scleritis denotes a higher likelihood of active vasculitis • When PUK is ass w/ scleritis there exists worse ocular and systemic outcome than when there is scleritis alone. • Complications include Severe Astigmatism, Secondary bacterial infection , perforation, cataract ,glaucoma • Cataract and glaucoma may occur as a result of the inflammatory process or the use of corticosteroids
SYSTEMIC ASSOCIATIONS 50% of patients with PUK may have an associated systemic disease with the large majority being the collagen vascular diseases (CVDs). Rheumatoid arthritis (RA) is most common CVD ass w/ PUK, likely due to its high prevalence in the population (affecting 2.5–3% of adults). Wegener’s granulomatosis and other ANCA-associated vasculitides, although relatively rare, are an important cause of PUK.
• Generally, patients with RA-associated PUK have an established advanced systemic diagnosis • However, in up to 25% patients, PUK may be the initial presenting feature of a potentially lethal undiagnosed systemic vasculitis, highlighting the importance of a thorough systemic evaluation.
General Examination 0f The Head And Extremities In Peripheral Ulcerative Keratitis Clinical Finding Associated Systemic Diseases Saddle Nose Deformity Relapsing Polychondritis, WG Auricular Pinnae Deformity Relapsing Polychondritis Nasal Mucosal Ulcers WG Oral/Lip/Tongue Mucosal Ulcers SLE, Sjogrens Syndrome Facial "Butterfly" Rash SLE Alopecia SLE, Progressive Systemic Sclerosis, Relapsing Polychondritis Hypo/Hyperpigmentation (Scalp, Face) PSS
Clinical Finding Associated Systemic Diseases Loss Of Facial Expression Rosacea, PSS Facial Telangiectasias Rosacea Rhinophyma All Vasculitic Syndromes Facial/Arms/Legs Rashes, Ulcers PSS Facial/Arms/Legs Taught Skin GCA Temporal Artery Erythema/Tenderness PSS, SLE, GCA, Sjogrens Syndrome Raynaud’s Phenomenon (Fingers) All Vasulitic Syndromes Ulcers In Fingertips RA, SLE, WG, Churg Strauss, PAN Subcutaneous Nodules In Arms And Legs All Vasculitic Syndromes
DIAGNOSTIC WORK-UP • A thorough ocular history, contact lens wear, current and previous medication for any diseases, trauma, or surgery • A comprehensive Systemic review to r/o systemic diseases ass w/ PUK • A complete ophthalmic examination to exclude local pathologies is critical.
Tests to exclude infectious etiologies Corneal scrapings and subject to 1. Gram Stain , KOH 2. Culture 3. Limulus Lysate Assay  Rapid and reliable method  Detect minute amounts of Gram Negative endotoxin  Prompt selection of appropriate anti microbial
LABORATORY INVESTIGATIONS 1. CBC , ESR 2. RF( +ve in 80 % patients with RA) 3. Angiotensin Converting Enzyme ( Sarcoidosis) 4. Anti Nuclear Antibody(ANA) (SLE ,RA) 5. Antibody to Double Strand DNA (anti –ds DNA)(SLE) 6. Antibody to small nuclear ribonucleoprotein (anti-RNP )(SLE) 7. Anti neutrophil cytoplasmic antibodies (ANCA ) c-ANCA in WG 8. Anti CCP 9. Urine Analysis 10. HBsAg 11. FTA –ABS 12. CXR 13. Sinus CT
14 . Biopsy of the bulbar conjunctiva adjacent to the ulcerating cornea should be considered, especially in suspected autoimmune associated PUK. Available modalities for studying the specimens include Light and electron microscopy Immunoprobes Polymerase chain reaction (PCR)
LABORATORY TESTS FOR SUSPECTED SYSTEMIC DISEASES IN PUK Systemic Diseases Laboratory Tests Systemic lupus erythematosus ESR, ANA (anti-dsDNA, anti-Sm) IgG, Complement, Cryogl Rheumatoid arthritis ESR, RF, ANA(anti-RNP), CIC, Complement, Cryog, joint X-rays Progressive systemic sclerosis ESR, ANA (anti-centromere, anti-Scl-70), RF, IgG, CIC Sjoren’s syndrome ESR, ANA (anti-RO, anti-La), CIC, RF, Cryo, IgG, IgA, IgM, sialography
Systemic Diseases Laboratory Tests Polyarteritis nodosa ESR, HbSAg, IgG, Cryog,complement, CIC, angiography Churg-Strauss ESR, WBC/Eosinophil count, IgE, CIC, Chest X-ray Relapsing polychondritis CIC, Complement Wegener’s granulomatosis ESR, IgA, IgE, RF, ANCA, CIC, sinus and chest x-ray, BUN, creat Giant cell arteritis ESR, CRP, CIC, IgG
• Infectious causes need to be excluded by the appropriate culture techniques • If infections are suspected- Antimicrobial therapy administered • Prophylactic topical antibiotics - prevent secondary infections ENHANCED LUBRICATION • Dilutes effect of inflammatory cytokines in preocular tear film • Many rheumatoid patients have KCS as a manifest of 2◦ Sjogren syndrome Melting stops or slows down if epithelium made to heal by Lubricants, Pathching, Bandage Contact lens Medical Treatment
Topical Collagenase Inhibitors • Sodium citrate 10 % • Acetylcysteine solution 20 % • Medroxy progesterone 1% Systemic Collagenase inhibitors • Tetracycline
Topical corticosteroids • considered as initial therapy in milder, u/l cases (typically when not associated with a systemic CVD) • Useful in mild cases of RA-associated PUK • May be harmful in a subset of vasculitic PUK because they inhibit new collagen production • Not effective in WG, microscopic polyangiitis, Churg–Strauss syndrome, and PAN • In these cases, corticosteroids may promote progression and even enhance perforation and thus must be used judiciously.
Systemic corticosteroids • Oral prednisone 1 mg/kg/day- very commonly used for the a/c management of more severe cases of PUK. • If there is progression, pulsed methylprednisolone (0.5-1.0 g) for 3 consecutive days may be effective. • Corticosteroids alone may be inadequate to control the progressive ocular disease process • Prolonged use of corticosteroids - significant systemic side-effects necessitating alternative steroid-sparing immunosuppressive agents.
Systemic immunosuppressants • Immunomodulatory agents, such as methotrexate, azathioprine, or cyclosporin A, mycophenolate mofetyl and Cytotoxic agents like cyclophosphamide have demonstrated clinical efficacy for inflammatory eye disease Indications for IMMUNOSUPPRESSION in PUK 1. ass w/ potentially lethal systemic vasculitic syndromes such as PAN, RA, SLE, PSS, Sjogren’s syndrome,WG, allergic angiitis of Churg-Strauss,GCA 2. ass w/ necrotizing scleritis and vasculitis confirmed by histopathologic analysis of ocular tissue. 3. B/l and /or progressive Mooren’s ulcer. 4. unresponsive to aggressive conventional medical and surgical therapy
• Studies suggest that cyclosporin A with dosages in the range of 2.5 mg/kg/day may be a reasonable initial choice for In idiopathic PUK, (especially if nephrotoxicity is not a concern) • In cases of PUK ass w/ WG , necrotizing scleritis or Severe or rapidly progressive PUK associated with other CVDs First-line therapy- Cytotoxic immunosuppressives, such as cyclophosphamide (2 mg/kg/day), together with corticosteroids (oral or pulse intravenous) Maintenance- oral or SC methotrexate(7.5 -12.5 mg /week)
Drug MOA Disease Dosage SIDE EFFECTS MONITOR Azathioprine Prodrug: 6 Mercaptopurine ,Purine nucleotide analog Blocks RNA ,DNA &protein synthesis, S phase specific Rheumatoid arthritis, WG, and Relapsing polychondritis (2nd Choice) 1-3 mg/kg/day ORAL Bone marrow suppression (reversible), increased risk of malignancies( NHL) LFT,Absolute platelet count Cyclophospha mide Alkylating agent , DNA to DNA cross linking , DNA miscoding WG and PAN (1st Choice), Rheumatoid arthritis 2 mg/kg/day ORAL/IM Bone Marrow Depression Blood Counts,LFT Methotrexate Inhibits DHFR, Bloocks DNA &RNA synthesis &repair , S phase specific Rheumatoid arthritis (1st choice), Maintenance for WG 7.5-12.5 mg/wk p.o. or IM Hepatotoxicity, cytopenia,int erstitial pneumonia CBC, LFT every 2 weeks for 1st month ,then every 4- 6 weeks, RFT Cyclosporine Inhibits IL 2 and othe rproinflammator y cytokines by inhibiting NF –AT transcription factor for signalling Idiopathic (1st Choice), Rheumatoid arthritis, Relapsing polychondritis (2nd Choice) 2–2.5 mg/kg Twice Daily Transient increase in creatinine (50 ),gingival hyperplasia (40 %),hirsuitism, nephrotoxicit y, HTN RFT, LFT, BP every 2 weeks Mycophenolate mofetil Selectove inhibitor of IMP Dehydrogenae , inhibits de novo synthesis , decreasing DNA synthesis Alternative to Azathioprine 1 g Twice Daily, with Max of 1.5 g Twice Daily Hepatotoxicity , leukopenia CBC, LFT
Biological agents • The efficacy of Biologic agents such as the anti-tissue necrosis factor (TNF) and anti-B cell monoclonal antibodies in the treatment of RA, WG, spondylo-arthropathies, inflammatory bowel disease as well as other systemic vasculitis suggest that they have a role. • Systemic therapy should be continued for a period of 6 months to 1 year after initial control of inflammation has been achieved
Drug Disease Dosage Biologic agents Anti-TNF Inflixamab RA, Crohn’s disease RA: 3 mg/kg IV infusion at 0, 2, 6 wk then every 8 wk. Crohn’s diseases 5 mg/kg 0, 2 and 6 wk then every 8 wk. Dosage may be increased interval of treatment reduced Adalimumab Wegener’s granulomatosis, RA 40 mg s.c. every 4 wk Anti CD22 (B cell) Rituximab WG: 375 mg/m2 once weekly for 4 wk. RA: Two 1000 mg IV infusion separated by 2 wk, preceeded by 30 min with 100 mg of IV prednisolone Dosage maybe increased interval of treatment reduced
Surgical Treatment Surgical treatments mainly to maintain the integrity of the globe and are typically palliative. Conjunctival resection • Temporarily remove the limbal source of local cellular mediators and collagenases important in progression of the disease process • May be of great diagnostic help Patch graft • When a perforation is too large for tissue adhesive to seal the leak, some type of patch graft will be necessary. • This may range from a small tapered plug of corneal tissue to a penetrating keratoplasty Tissue adhesive Application of Isobutyl cyanoacrylate glue , forms a biological barrier b/w host cornea and the reepithelializing conjunctiva and the immune
Superficial lamellar keratectomy • arrest the inflammatory process and allow healing Rehabilitative surgical therapy • Initial lamellar tectonic grafting followed by central penetrating keratoplasty • In advanced cases • LKP is the most widely practiced surgery – in 2 stages • For an ulcer < 1/2 circle of limbus and the central 7-8 mm of cornea uninvolved, crescent shaped lamellar graft can be used. • For an ulcer > 2/3 of a circle of limbus where the central 7-8 mm of cornea is intact, a doughnut shaped lamellar graft is recommended.
1st stage • Lamellar flap that encloses corneal pathology –microkeratome • Flap lifted to ensure underlying stroma clear, replaced 4-6 week interval • Flap stabilizes and partially adhere 2nd stage • Trephine over previously made flap to a depth slightly below initial lamellar dissection • 1mm flap rim left behind, Flap removed • Donor tissue transferred to host bed  Spontaneously adhere  10-0’nylon sutures  Bandage Contact Lens Femto second laser can be used for making the flap
• Amniotic Membrane transplantation- preserve the integrity of the globe • Cyanoacrylate adhesive in conjunction with immunosuppressive therapy may be used in patients with impending perforation and may delay the need for tectonic corneal surgery. • Conjunctival flaps  control corneal melting in difficult to manage microbial keratitis,  but best avoided in immune mediated disease  Bringing conjunctival vasculature closer to area of corneal disease accelerate melting
COURSE AND OUTCOME • The course, duration, and outcome are variable and dependent on the underlying cause of the PUK and on prompt and appropriate management. • Many patients with mild or moderate PUK may maintain good vision if the inflammatory process is rapidly controlled. • The prognosis is more guarded when PUK is ass with a systemic CVD. • Significant visual loss and ocular morbidity may develop with corneal perforation. • Both ocular and systemic prognosis is more guarded when there is concomitant scleritis, especially necrotizing scleritis
Mooren’s ulcer • strictly a PUK, with no associated scleritis. • Bowman published the first report of Mooren’s ulcer in 1849. • In 1854, McKenzie described it as ‘c/c serpiginous ulcer of the cornea or ulcus roden.’ • The disorder was named as Mooren’s ulcer after Dr Mooren, who was the first to clearly describe this insidious corneal problem and define it as a clinical entity
• Mooren’s ulcer can occur in all age groups, but the vast majority present b/w 40 and 70 years. • Disease can occur in either sex, but men outnumber women. • U/l involvement is more common • Interpalpabral limbus is involved most often, followed by the inferior and then the superior limbus. • occurs in complete absence of any diagnosable systemic disorder that could be responsible for progressive destruction of the cornea.
• rare, degenerative, superficial ,idiopathic disease • painful, rapidly progressive, chronic ulcerative keratitis that begin peripherally and progresses circumferentially , then centripetally • single or multi-centric crescent shaped peripheral corneal ulcer with a characteristic steep, infiltrated, undermined, or overhanging edges
Healing: Corneal epithelialization and vascularization associated with scarring and thinning. Advanced cases: most of the cornea is lost leaving behind a central island surrounded by area of grossly thinned, scarred, and vascularized tissue.Corneal perforation is uncommon.
Epidemiology Wood and Kaufman described two clinical types of Mooren’s ulcer. Limited type(typical or benign Mooren’s ulcer) • U/l with mild to moderate symptoms, • generally responds well to medical and surgical treatment. • occur in older patients Atypical or malignant Mooren’s ulcer • B/l although both eyes may not be affected simultaneously • relatively more pain and generally a poor response to therapy. • younger patients • progresses relentlessly and is more likely to result in corneal perforation.
Watson, based on clinical presentation and anterior segment fluorescein angiographic findings, divided Mooren’s ulcer into three distinct varieties. U/l Mooren’s ulceration • painful progressive corneal ulceration in elderly • Ass w/ nonperfusion of the superficial vascular plexus of the ant segment B/l aggressive Mooren’s ulceration • occurs in young patients • progresses circumferentially, then centrally in the cornea. • Vascular leakage and new vessel formation, extending into base of ulcer. B/l indolent Mooren’s ulceration • usually in middle-aged patients • presenting with progressive peripheral corneal guttering in both eyes, with little inflammatory response
Pathogenesis • Although etiology unknown, precipitating factors include accidental trauma or surgery and exposure to parasitic infection(incidence is higher in areas of endemic infection) • Ass w/ infections- helminthiasis, hep C, herpes simplex and zoster. • Characteristics of an autoimmune process • decrease in number of suppressor T cells relative to no of helper T cells • elevated IgA levels • Higher circulating IgG antibodies and immune complexes to human corneal and conjunctival epithelium. • Ratio of CD4/CD8 cells and B7-2/antigen-presenting cells higher • Ass w/ HLA-DR17
• Antibodies to an auto-antigen that exists in corneal stroma. • The antigen, known as ‘cornea-associated antigen’, has an amino acid sequence identical to that of calgranulin C of neutrophils. • No change from normal vascular architecture except an extension of new vessels into the ulcer. • Trauma or infection alter normal corneal antigenautoimmune response • Altered antigen taken up by macrophages(APC),  T-cell activation, differentiation, and proliferation. • Lymphocytes return to the conjunctival vesselsocular inflammation.
• Corneal edema from inflammation  easier diffusion of immune components into pericentral and central cornea from the limbal vessels • Thus cornea is damaged, liberating more altered corneal antigens that aggravate and perpetuate the process until the corneal stroma is completely destroyed • Molecular mimicry may be involved, stimulating an auto immune response. • Deposition of immune complex over peripheral cornea • Auto immune lysis of epithelium with release of collagenolytic enzymes.
• Grey infiltrates , which break down, forming small ulcers that spread and coalesce. • It destroys the epithelium and superficial stromal lamellae, forming a whitish overhanging edge, while the base quickly becomes vascularised.
SYMPTOMS • severe and persistent neuralgic pain. • Redness Photophobia Lacrimation • Decrease in visual acuity due to associated iritis, central corneal involvement and irregular astigmatism.
• The following stepwise approach to management is recommended 1.Topical steroids and antibiotics-Unilateral, mild-moderate cases 2. Conjunctival resection Conjunctiva is resected up to 2 clock hours on either side to bare sclera and extend 3–4 mm from the limbus. Excision of a 4-7 mm strip of adjacent conjunctiva may prove successful by eliminating conjunctival sources of collagenase , proteoglycanase and other inflammatory mediators. 3. Topical acetylcysteine 10% and L-cysteine (0 .2 molar), topical cyclosporine
4. In case of Perforation: • ulcer debridement with cyanoacrylate adhesive and soft contact lenses. • Lamellar keratoplasty with IV methotrexate– halt the process 5.Topical IF 2 alpha, Infliximab 6. Systemic immunosuppression-Bilateral or progressive Mooren's ulcer that fail w/therapeutic steroids and conjunctival resection will require systemic cytotoxic chemotherapy 5.Additional surgery: Keratoepithelioplasty Lamellar keratectomy
• A stepwise approach to treatment of Mooren's ulcer is used. • Treatment proceeds from topical corticosteroids to conjunctival excision with or without lamellar keratectomy and, finally, to systemic immunosuppression. • However, if a patient presents with b/l simultaneous ulcer or advanced disease or progresses rapidly, little time is spent waiting for a therapeutic response to topical therapy, and treatment consisting of conjunctival excision with systemic immunosuppression is undertaken.
ROSACEA KERATITIS(acne rosacea) • c/c acneiform inflammatory disorder chiefly affecting skin and eyes • unknown etiology but may be related to overexpression of cathelicidin antimicrobial peptides. • Cathelicidins cause neutrophil recruitment, angiogenesis, and cytokine release, which may contribute to the inflammatory skin changes • sebaceous gland dysfunction of the face, neck, and shoulders • More common in women in 4th decade • More severe manifestations occur in men • Alcohol can contribute to a worsening of this disorder because of its effect on vasomotor stability
CLINICAL PRESENTATION • More common in fair-skinned individuals, it may simply be more difficult to diagnose in people with dark skin. • Skin manifestations precede Corneal manifestations except in 10% of cases • Facial lesions consist of telangiectasias, recurrent papules and pustules, and midfacial erythema • Characteristic malar rash with unpredictable flushing episodes, ass w/ consumption of alcohol, coffee, or other foods • Hypertrophic cutaneous changes occur late in the disease process-Rhinophyma
• Excessive sebum secretion with frequently recalcitrant c/c blepharitis. • Eyelid margin telangiectasia • Meibomian gland distortion, disruption, and dysfunction, which can lead to recurrent chalazia. • c/c conjunctivitis, marginal corneal infiltrates ,sterile ulceration, episcleritis, or iridocyclitis. • If properly treated, these lesions can resolve with few sequelae. • Repeated bouts of ocular surface inflammation can cause corneal neovascularization and scarring
CORNEA  Affects inferior quadrant of cornea  Punctate epithelial erosions  Vascular invasion of the peripheral cornea with subepithelial infiltrates  Progresses centrally and circumferentially –ulceration ,scarring and perforation • Limbal involvement – Nodular conjunctivits, Nodular scleritis
MANAGEMENT • Systemic tetracyclines as the mainstay of therapy. • Tetracyclines have anti-inflammatory properties that include suppression of leukocyte migration, reduced production of NO and ROS, inhibition of MMPs, and inhibition of phospholipase A2 and reduce irritative FFA and diglycerides by suppressing bacteriallipases. • With time, oral therapy with doxycycline or minocycline can be tapered. • Oral tetracycline 250 mg QID for 6 – 8 weeks • After that dosage to be tapered by 250 mg per week • Tetracycline 250mg OD for a year before discontinuing
• Topical metronidazole 0.75% gel, metronidazole 1% cream, or azelaic acid gel 15% to the affected facial areas reduce facial erythema. • PUK can be ass w/ infectious agents in rosacea, or it may have a sterile inflammatory etiology. • Once it is ascertained that PUK is noninfectious, topical corticosteroids, used in reducing sterile inflammation and enhancing epithelialization of the cornea. • In advanced cases with scarring and neovascularization, conservative therapy is generally recommended. • Penetrating keratoplasty is a high-risk procedure in rosacea patients; it may result in a poor prognosis if the ocular surface is severely compromised. • Light-pulse treatment may help reduce eyelid erythema.
RHEUMATOID ARTHRITIS • Commonest systemic association • Involves both eyes in 30% cases • Tends to affect during late and advanced vasculitic phase • Ass w/ all types of anterior scleritis including Diffuse, nodular, necrotizing scleritis with inflammation and necrotizing scleritis without inflammation (scleromalacia perforans), as well as posterior scleritis
• Corneal findings  PUK  Sclerosing keratitis(gradual thickening and opacification of corneal stroma adjacent to site of scleritis)  Acute stromal keratitis  Limbal guttering  Keratolysis  Keratoconjunctivitis sicca
PATHOGENESIS • Immune complex deposition in peripheral cornea and limbal vessels • Chemotaxis of inflammatory cells There are 2 types of PUK in RA 1)Paracentral 2)Peripheral Paracentral keratolysis • In patients with severe dry eye • Mechanism – Altered epitheliumInflammatory mediators enter the stromaStimulate a sterile keratolysis and a T cell infiltrate • Progresses both centrally and circumferentially • Unresponsive to topical therapy
2.Peripheral keratolysis • There is an obliterative Microangiitis at the level of limbal vascular arcades • Deposition of Immune complexes in limbal vessels • Causes an immune microangiopathy and obliterative microangiitis • leakage of inflammatory cells and proteins • collagenases and proteases are released by neutrophils • corneal melting
Rheumatoid polyarthritis suspected in an HIV patient with scleritis ,peripheral ulcerative keratitis, and anterior uveitis by Domngang Noche C1, Singwé-Ngandeu M2, Bella AL2 • 37/F • K/c/o AIDS on ART for 3 years • H/o c/c polyarthropathies x past 6 months • Presented with RE- nodular scleritis LE-PUK,necrotizing scleritis,granulomatous ant uveitis • Ophthalmic workup- negative for opportunistic infections and potential causes of scleritis and PUK • Patient unresponsive to topical antibacterial and anti-inflammatory treatment
• Sulfasalazine started • Ocular lesion resolution and articular swelling improvement observed in <6 weeks • Based on American College of Rheumatology classification criteria, and considering the good response to the treatment (sulfasalazine), diagnosis of Rheumatoid Arthritis with ocular involvement was made in the absence of confirmatory lab tests result.
Necrotising Scleritis and Peripheral Ulcerative Keratitis Associated with Rheumatoid Arthritis Treated with Rituximab. By Hardy S1, Hashemi K1, Catanese M1, Candil M2, Zufferey P2, Gabison E1, Guex-Crosier Y1. • 3 cases of PUK ass/ w/ RA • 2 patients - necrotising scleritis • In the 2 patients who received the induction therapy with rituximab , good initial therapeutic response observed - stabilized from an ocular standpoint as compared to patient on only high dose corticosteroid • The patient who received only corticosteroid developed massive colon perforation as well as acute renal insufficiency early on in the Rx course • In patients receiving rituximab, eventually the drug had to be switched to other molecules, either due to other systemic symptoms from the disease itself or due to adverse effect of this treatment. • This study contributes to other reports stating that rituximab can be an effective treatment for refractory ocular complications in RA, at least as an induction therapy.
WEGENER’S GRANULOMATOSIS(Granulomatosis with Polyangiitis) • Multisystem necrotizing granulomatous vasculitic disease • Common ophthalmic manifestations-Usually bilateral  Orbital involvement with proptosis  PUK  adjacent necrotizing scleritis  Sometimes conjunctivitis or episcleritis PUK • can be the initial clinical manifestation • May be the presenting or only sign of the disease. • Crescentic peripheral corneal ulcer that resembles Mooren’s ulcer • Scleral involvement is invariably present C/F from Moorens
HISTOPATHOLOGY • Lymphocytes and plasma cells predominate the substantia propria • Sclera and episclera may show a Granulomatous reaction with epithelioid cell and giant cells • Areas of active collagen degradation can be seen p ANCA testing is positive MANAGEMENT Systemic immunosuppression with cyclophosphamide and steroids
Peripheral Ulcerative Keratitis Associated with Granulomatosis with Polyangiitis Emerging Despite Cyclophosphamide, Successfully Treated with Rituximab. By Fujita Y1, Fukui S1,2, Endo Y1, Tsuji S1, Takatani A1, Shimizu T1, Umeda M1,3, Nishino A1,4, Koga T1,5, Kawashiri SY1,2, Iwamoto N1, Ichinose K1, Tamai M1, Nakamura H1, Origuchi T1,6, Ueki R7, Uematsu M7, Ishida K8, Abe K8, Kawakami A1. • 67/M • K/c/o GPA (right maxillary sinusitis, proteinase 3 ANCA positivity) • RE scleritis • A conjunctival biopsy specimen -neutrophil-predominant infiltration around the vessels without granuloma • Pulsed methylprednisolone and IV cyclophosphamide pulse therapy started.
• However, it was ineffective, and PUK newly emerged. • Promptly switched the patient from IVCY to rituximab, which controlled ocular disease • Amniotic membrane transplantation.
A case report of the orbit, ocular association and the lung in granulomatosis with polyangiitis: A diagnostic challenge. By Lu CW1, Liu XF1, Luan Y1, Lu CB2, Zhou DD3, Guo LM1, Sun YB1, Chen SN1, Wu YL1, Hao JL1. • 40/F • Progressive worsening pain, redness and gradual decrease in VA in the BE x 7 years • Previous therapeutic interventions - noncompliant topical and IV dexamethasone x 6 years. • H/o asymptomatic space-occupying lesion in the right middle lung, which was surgically removed 4 months back • 1 month later,h/o surgical removal of Left orbital mass • Diagnosed as PUK ass w/ GPA
• Corneal lesions were then treated bilaterally with cryotherapy and oral prednisone and cyclophosphamide were administered. • Following surgery, Condition of the eyes appeared to be stable. • 7 months later, the redness and pain of the right eye recurred, followed by a deep lamellar keratoplasty for the treatment of necrotizing scleritis. • The condition of 2 eyes was subsequently observed to be stable during the 9 month follow-up. • Thus,GPA must be considered in D/d of inflammatory conditions and tumors • Early diagnosis and an appropriate interdisciplinary approach to management, are required to decrease recurrence and morbidity in patients with GPA-mediated inflammatory ocular disease.
Successful Treatment Strategies in Granulomatosis With Polyangiitis- Associated Peripheral Ulcerative Keratitis. BYEbrahimiadib N1, Modjtahedi BS, Roohipoor R, Anesi SD, Foster CS. • Patient database of Massachusetts Eye Research and Surgery Institution was searched from 2005 to 2015 to identify patients of PUK w/ GPA. • 16 patients mean follow-up of 64 months duration • Rituximab and cyclophosphamide, either alone or in combination with other agents- most successful agents in controlling inflammation. • Rituximab in 11 patients with remission being achieved in all (100%) • Cyclophosphamide successfully controlled inflammation in 50% (5/10).
• 2 patients (2/5, 40%) who had achieved initial control on cyclophosphamide had flares of their PUK. • 2 patients (2/11, 18%) patients on rituximab had flares of scleritis and orbital inflammation but not PUK. • 2 patients, 1 in each treatment group, stopped treatment after achieving remission after 6 months of therapy but suffered disease recurrence within 2 months of treatment cessation. • Rituximab achieved a high rate of disease control in PUK patients with GPA and is the preferred agent in halting disease progression.
Surgical treatment of peripheral ulcerative keratitis and necrotizing scleritis in granulomatosis with polyangiitis. By Lu CW1, Zhou DD, Wang J, Hao JL • 70/M • progressively worsening pain and redness RE x 6 months, and decreasing VA x 1 month • Was misdiagnosed and treated as keratitis elsewhere, but pain had not decreased. • Diagnosis of necrotizing scleritis with PUK in GPA made • Surgery was performed with necrotic tissue removal, combined with cryotherapy and a conjunctival flap covering surgery. • Symptoms relieved after surgery, and the patient's ocular condition remained stable thereafter. • This case first demonstrates limited ophthalmic GPA (such as, PUK and necrotizing scleritis) that was not aggravated can be successfully treated by combined surgical interventions.
POLYARTHRITIS NODOSA  PUK morphologically similar to Mooren’s ulcer with the central overha nging edge may be present.  Usually associated adjacent necrotizing scleritis is present.
Rapid corneal thinning and perforated ulcerative keratitis in a patient with relapsing polychondritis by Lai THT1, Far N2, Young AL1,2, Jhanji V2,3. • 43 Male • K/c/o relapsing polychondritis w/ b/l scleritis, b/l swelling of pinna, saddle nose and tracheal stenosis. • Right eye pain and redness x 1 month. • O/E- 80% peripheral corneal thinning b/w 3 and 7 o'clock in RE • 1 week of oral corticosteroid use • The degree of corneal thinning worsened to 90% • Subsequently, topical cyclosporine 2% eye drops QID, oral doxycycline 100 mg BD oral vitamin C 2 g OD added • Corneal thinning improved to 60%.
• Patient rapidly tapered oral prednisolone against medical advice- returned with an a/c drop in vision in RE. • On Slit lamp -peripheral corneal perforation with iris prolapse. • Emergency repair with cyanoacrylate glue. • IV methylprednisolone 1 mg/kg body weight x3 days and 1 g/day and IVIG every 4 weeks. • 3 months postop, a well-formed anterior chamber with glue in situ.
Gout Keratitis: A Case of Peripheral Ulcerative Keratitis Secondary to Gout With a Review of the Literature. byYazdanyar A1, Rizzuti AE, Mechel E, Denisova K, Lazzaro DR. • 41/ M / k/c/o severe gout disease • Pain and redness of RE • O/E-2 areas of peripheral corneal thinning with overlying epithelial defects. Adjacent to these areas, reflective crystals identified in the corneal stroma. • AS-OCT demonstrated stromal corneal deposits. • Systemic workup was negative aside from an elevated serum uric acid level. • The patient was administered oral prednisone, allopurinol, and colchicine. • 2-month follow-up visit, the patient was asymptomatic and his corneal thinning had significantly improved. • Crystal deposition in the cornea is extremely rare and may be a cause of peripheral ulcerative keratitis.
Peripheral ulcerative keratitis associated with chronic malabsorption syndrome and miliary tuberculosis in a child byTarun Arora, Namrata Sharma,1 Adarsh Shashni, and Jeewan S. Titiyal • 16/F • Pain, redness, watering, and blurring of vision RE • O/E RE PUK and nodular scleritis. • Systemic examination-stunted growth, low BMI, enlarged axillary nodes. • Giardia cysts in the stool sample • Histopathology of axillary lymph nodes showed caseating necrosis s/o TB • Diagnosed as PUK with c/c malabsorption syndrome secondary to giardiasis and miliary TB • Oral metronidazole, anti-tubercular treatment, high protein diet and vitamin supplements were started. • Topical steroids were started for peripheral ulcerative lesions following, which the PUK resolved.
Peripheral Ulcerative Keratitis Secondary to Chronic Hepatitis B Infection. By Wei DW1, Pagnoux C, Chan CC. • 52/Female • RE eye pain and photophobia • Arthralgia, remnants of a maculopapular rash, subsequently facial numbness several weeks later. • BCVA in RE 20/80. • Slit-lamp examination -severe superior corneal thinning without infiltrate. • Corneal ulceration worsened until 10% of the cornea remained. • Laboratory workup was positive for rheumatoid factor and revealed significantly decreased C4 complement, and HBV serology was positive.
• Clinical history, examinations, and laboratory results suggest HBV-associated cryoglobulinemia and vasculitis. • Management included prednisone, cyclophosphamide, and mycophenolate mofetil for immunosuppression and tenofovir for HBV treatment. • Conjunctival resection and a glue patch were used to reduce inflammation and stabilize corneal melt. • BCVA improved after treatment was initiated. • Two years after initial presentation, her BCVA is 20/30, significantly improved from her vision at presentation.
Peripheral Ulcerative Keratitis Associated with HCV-Related Cryoglobulinemia. By Coelho P1, Menezes C2, Gonçalves R1, Rodrigues P1, Seara E1. • 36/M • H/o Hepatitis C and IV illicit drug abuse • moderate pain ,redness and photophobia in RE. • O/E BCVA RE - 20/30 • slight discomfort with eye movement. • Ring shaped peripheral corneal ulcer, with a dense white stromal infiltrate sparing the limbus, plus an adjacent area of mild anterior diffuse nonnecrotizing scleritis. • No intraocular inflammation was present. • Posterior segment was normal.
• oral prednisolone (1 mg/Kg/day) with gradual tapering. • A dramatic clinical response occurred, with complete resolution of the clinical condition • Systemic analytic workup aiming at autoimmune and infectious diseases was negative, except for high HCV-viral load and the presence of type 2 cryoglobulins.
Contact lens wear • reduces the amount of O2 available to the cellular components of cornea • Less tear flow under contact lens than which will normally pass over cornea • may increase the temperature of the cornea • the insertion and removal of lenses may produce regions of microtrauma to the corneal surface, limbus, and adjacent conjunctiva. • Soft contact lenses worn overnight,risk of a corneal infection is increased. • Fequently, patients will not change or clean their contact lens storage cases, which can harbor bacteria and other microorganisms. • certain systemic diseases, such as diabetes and other diseases that slow tissue healing, can increasthe risk of ocular infections. • Dry eye and lagophthalmos serve to compromise the ocular surface. When these conditions are present in an individual who also wears contact lenses, the risk of microbial keratitis increases significantly.
Mooren Ulcer in a Child Wearing Orthokeratology Contact Lenses. BY Kim J1, Kim MK, Wee WR, Oh JY. • 10/M • Referred for progressive peripheral corneal ulcer RE, despite intensive use of fortified antibiotic eye drops. • H/o overnight orthokeratology lenses x 4 months before presentation of corneal ulcer. • No other history of ocular or systemic trauma and disorders. • Microbiological tests of the lesion were negative. • Systemic evaluation showed no sign of rheumatologic disease. • Under a diagnosis of Mooren ulcer, the patient was treated with topical and systemic corticosteroids. • After four weeks of treatment, the patient's symptoms rapidly disappeared, and corneal ulcer was healed. • Vision recovered to normal with the correction of with-the-rule astigmatism.
Case-Control Pilot Study of Soft Contact Lens Wearers With Corneal Infiltrative Events and Healthy Controls. By Richdale K1, Lam DY2, Wagner H3, Zimmerman AB4, Kinoshita BT5, Chalmers R6, Sorbara L7, Szczotka-Flynn L8, Govindarajulu U1, Mitchell GL4. • Purpose :To assess risk factors associated with soft contact lens (SCL)- related corneal infiltrative events (CIEs). • Single-visit, case-control study • 30 Cases defined as current SCL wearers w/ a symptomatic CIE. • For each case, 3 age- and sex-matched controls were enrolled(90 controls ) • 13 to 31 years of age • Subjects supplied a recent health history ,completed the Contact Lens Risk Survey , a standardized scripted medical interview,, and underwent an ocular examination. • Microbial culturing of the ocular surface, SCL, and lens storage case was conducted for all cases.
• CIE diagnosis included contact lens-associated red eye, infiltrative keratitis, and contact lens peripheral ulcer. • Subjects with symptomatic CIEs more likely to harbor substantial levels of gram-negative bioburden on the ocular surface and contact lens. • Significant risk factors for developing a CIE were overnight wear of SCLs, use of multipurpose solution, rinsing SCLs with water, lens storage case older than 6 months, previous "red eye" event, use of ocular drops in the past week, and illness during the past week. • A larger sample size is needed to determine relationships between patient-reported behaviors and exposures, microbial bioburden, and CIE development.
Peripheral Ulcerative Keratitis following Laser in situ Keratomileusis. By Burkholder BM1, Kuo IC1. • 28/M underwent LASIK • h/o glomerulonephropathy, not disclosed prior • Developed severe postop PUK soon after surgery • Within a week of surgery developed diffuse lamellar keratitis (DLK) not contiguous with PUK • Microbiologic evaluation of the flap interface disclosed no organisms, and no epithelial ingrowth was found. • Both PUK and DLK resolved with topical and oral steroid therapy, and the patient's induced refractive error improved over the 12 months following LASIK. • This case demonstrates importance of diligent history taking to exclude autoimmune disorders or atypical postinfectious sequelae prior to proceeding with keratorefractive intervention.
GIANT CELL ARTERITIS
Infectious Disorders of the Peripheral Cornea • As peripheral cornea is adjacent to the rich vascular supply of the limbus, the limbal lymphatic tissue, and inflammatory cells, microbial keratitis maybe thought to be less frequent than in the central cornea. • However, other factors increase the likelihood of a peripheral corneal infection. Bacterial keratitis • occurs in the inf third of the cornea, could be secondary to corneal exposure. • This should especially be considered in an individual who has suffered multiple ocular infections. • Significant pain • Markedly hyperemic bulbar conjunctiva • Quicly progress to perforation –w/in 24-48 hours • May be Ass w/ uveitis- dense flare and cells , hypopyon
Common organisms • St aureus ,St epidermidis • Streptococcus pneumoniae and other strs • Pseudomonas aeruginosa (soft contact lens wearers) • Enterobacteriaceae(proteus, Enterobacter) • Moraxella spp Uncommon organisms – Neisseria spp – Listeria – Mycobacterium spp – Nocardia spp – Non-spore-forming anaerobes – Corynebacterium spp – Haemophilus influenzea – Chlamydia trachomatis Intact coneal Keratitis • N. gonorrhoeae • N. meningitidis • C. diphtheriae • H. influenzae • Shigella
Staphylococcal ulcer • Well defined • Gray-white stromal infiltrate Streptococcal • Infiltrate purulent • Severe AC reaction • Hypopyon formation Pseudomonas • Rapidly progressive • Suppurative • Necrotic infiltrate • Ass w/ a hypopyon and mucopurulent discharge
Presentations: • Rapid onset of pain • Decreased vision • Photophobia • Discharge • Eyelid edema Signs: • Conjunctival injection • White stromal infiltrate associated with an overlying epithelial defect and secondary anterior uveitis • Enlargement of stromal infiltration associated with stromal oedema • Severe infiltration and hypopyon formation • Corneal perforation and endophthalmitis in neglected cases
Investigations Corneal scraping and culture • Blood agar : most bacteria & fungi except Neisseria, Hemophilus & Moraxella • Chocolate agar : Neisseria, Hemophilus, Moraxella • Sabouraud dextrose agar : fungi • Non nutrient agar : Acanthamoeba • Brain Heart Infusion : Streptococci and Meningococus • Löwenstein-Jensen : Mycobacteria and Nocardia Staining • Gram : Bacteria, fungi, Microsporidia • Giemsa : Bacteria, fungi, Acanthamoeba, Microsporidia • Calcofluor white : Acanthamoeba, fungi, Microsporidia • Acid-fast stain : Mycobacterium, Nocardia Conjunctival swabs : Severe cases and corneal scraping is negative Contact lens cases
• Rx 1.Fortified topical /periocular injection of antibiotics  GRAM POSITIVE (Staphyloccus, Strep. Pneumoniae)-semi synthetic Penicillin, Vancomycin,Cephalosporin,Bacitracin  GRAM NEGATIVE (Pseudomonas aeruginosa)-Gentamicin, Tobramycin, Carbenicillin, Colistin, Polymyxin B Monotherapy; – Fluoroquinolone (Ciprofloxacin 0.3% or ofloxacin 0.3% 2.Mydriatic –Cycloplegic Prevent synechiae due to accompanying Seveere uveitis 3.Anti-Collagenase compounds- decrease corneal melting 4. Lateral, medial, or both types of tarsorrhaphy may be required to protect the ocular surface and prevent subsequent episodes of infectious keratitis.
• Microbial keratitis from syphilis or TB can produce peripheral corneal changes • Syphilis ass w/ a deep corneal inflammation with deep vessels in the peripheral cornea. • TB produces an a/c inflammation that is more commonly u/l and involves peripheral sectoral portions of the anterior cornea.
Ocular syphilis masquerading as bilateral peripheral ulcerative keratitis. By Vignesh AP1, Srinivasan R1, Vijitha S1. • 55/M • recurrent pain and redness BE X 2 months. • BE had b/l peripheral crescent-shaped corneal ulcers suggestive of PUK. • The patient was started on topical steroids elsewhere, but lesion was not showing any signs of healing. • A series of investigations performed- positive VDRL and fluorescent treponemal antibody absorption tests. • Started on systemic penicillin, as well as topical steroids. • The response to the treatment was good and the ulcer began to heal.
Fungal Keratitis • Less common than BK • 5%-10% of corneal infections • May have devastating effects • severe stromal necrosis • may enter AC by penetrating intact Descemet membrane • difficult control because poor penetration of antimycotic agents. • Filamentous fungal : Asprgillus vs Fasarium • Non Filamentous or Yeast : Candida Predisposing factors : c/c ocular surface disease, long-term use of topical steroids,Contact lens wear, systemic immunosuppression, diabetes,vegetable matter
Presentation Gradual onset of FB sensation, Photophobia,Blurred vision,Discharge Signs Filamentous keratitis • Greyish-white infiltrate with indistinct feathery margins. • Often surrounded by finger-like satellite stromal infiltrates Candida keratitis •Yellow-white infiltrate • dense suppuration • similar to bacterial keratitis
Investigations • Staining - Gram and Giemsa staining - Periodic acid-Schiff (PAS) • Culture - Sabouraud dextrose agar - on blood agar or in enrichment media. - contact lenses • Corneal biopsy - is indicated in the absence of clinical improvement after 3–4 days - if no growth develops from scrapings after a week.
Management • Removal of the epithelium • Topical treatment : hourly for 48 hours - Candida : amphotericin B 0.15% or econazole 1%;or natamycin 5%, or fluconazole 2%, or clotrimazole 1% - Filamentous infection : natamycin 5% or econazole 1%; or amphotericin B 0.15% or miconazole 1%. • A broad-spectrum antibiotic • Cycloplegia • Subconjunctival fluconazole • Systemic antifungals lesions are near the limbus, or for suspected endophthalmitis • Superficial keratectomy • Therapeutic keratoplasty
Herpes simplex and zoster • can develop peripheral corneal ulcers that do not exhibit typical dendrite formation. • Instead, they develop an irregular epithelium or epithelial defect over the affected area, followed by a region of stromal haze that assumes a crescent shape adjacent to the limbus. • The associated limbus is typically hyperemic and the eye is painful. • Herpes simplex and zoster may also produce a sterile or an infected neurotropic ulcer • Therefore, corneal sensation should always be tested in patients who experience little pain in association with a corneal ulcer. • Treatment is directed at healing the ulcer and protecting the ocular surface with a tarsorrhaphy. • A scleral contact lens may protect the corneal surface once the ulcer is healed.
Actinobacillus actinomycetemcomitans Keratitis After Glaucoma Infiltration Surgery: A Clinical Report and Literature Review. BY Hong J1, Xu J, Cao W, Ji J, Sun X • 56/M / Long-standing h/o open-angle glaucoma BE • h/o ocular pain, redness, blurred vision RE x 12 weeks • h/o Glaucoma infiltration surgery RE x 6 months ago. • 3 months postop, developed peripheral corneal stromal opacities ass w/ a white, thin, cystic bleb, conjunctival injection. • These opacities grew despite topical treatment w/ topical tobramycin, levofloxacin, natamycin, amikacin, metronidazole eye drops. • Multiple corneal scrapings revealed no organisms • Aerobic, anaerobic, fungal, or mycobacterial cultures negative
• RE developed a severe purulent corneal ulcer with a dense hypopyon and required corneal transplantation. • Histopathologic analysis and 16S ribosomal RNA PCR sequencing revealed A actinomycetemcomitans as the causative organism. • Postop, treatment was initiated with topical levofloxacin and cyclosporine, as well as oral levofloxacin and cyclosporine. • Graft and host corneal transparency were maintained at the checkup 1 month after surgery. • Although it is a rare cause of cornealdisease, A actinomycetemcomitans should be suspected in patients with keratitis refractory to topical antibiotic therapy • Delay in diagnosis and appropriate treatment can result in vision loss.
Differential Diagnosis 1.Marginal keratitis ass w/ blepharitis 2.Pellucid marginal degeneration 3. Terrien’s marginal degeneration 4.Furrow degeneration
Local inflammatory causes • Staphylococcus marginal ulcers, which begin with an infiltrate adjacent to the limbus separated by a characteristic clear zone with progression to ulceration • Rosacea-associated blepharo-conjunctivitis which may result in a peripheral keratitis with or without a clear zone. Non inflammatory cause of thinning of the peripheral cornea Terrien’s marginal degeneration, pellucid marginal degeneration, and marginal furrow can be distinguished from PUK due to lack of inflammation and typically an intact epithelium
Pellucid marginal corneal degeneration Unknown etiology  Inferior peripheral corneal thinning and protrusion Point of greatest protrusion is just above the area of maximal thinning Absence of inflammation The involved area is clear, and usually no iron line occurs central to it. Marked against-the-rule astigmatism-Decreased vision Rx-  Contact lenses  Penetrating Keratoplasty  Wedge resection  Lamellar Tectonic grafts  Collagen Cross linking
Terrien’s degeneration • Uncommon idiopathic thinning and ectasia of superior peripheral cornea • Arcuate lipid deposition and vascularisation in pts aged >40 yrs • 75% Male After 4th decade ,Usually B/l • Initially asymptomatic • Lack of inflammation or epithelial defect • Presence of sup vascularization and advancing linear deposition of lipid • Slow progressive course • No known systemic disorders occur with terrien’s marginal degeneration • Associated with VKC
• Mild, fine yellow white ,punctate, sub-epithelial or anterior stromal opacities • Peripheral, superficial, fine vascular pannus which start superiorly, spread circumferentially and progresses over years • Linear sub-epithelial opacity at the advancing edge • The thinning slowly begins between the limbus and the line of lipid deposition and is separated from limbus by clear zone • Typically, a steeper sloping of the cornea occurs at the advancing edge. • Gradual vision disturbance due to increasing corneal astigmatism • Pseudopterygia in long standing cases With increased corneal thinning -> ectasia Can perforate after trauma
HISTOPATHOLOGY • Fibrillar degeneration -Bowman’s Layer and stromal lamellae • Cornea thinner with appearance of a subepithelial pannus Rx • Spectacles • Contact lens-scleral lenses or soft lenses with RGP piggybacking • Tectonic corneal grafting • Topical lubricants and mild topical steroids
Blood derived eye drops for the treatment of cornea and ocular surface diseases. By Giannaccare G1, Versura P2, Buzzi M3, Primavera L2, Pellegrini M2, Campos EC2. • Use of blood derived eye drops for the Rx of ocular surface disorders, mainly dry eye disease, persistent corneal epithelial defect, corneal ulcer, ocular surface burn, recurrent corneal erosion and limbal stem-cell deficiency has become increasingly popular in recent years. • MOA -stimulation of cellular proliferation and migration by supplying an active mixture of growth factors and cytokines at the ocular surface, thus mimicking function of the lacking natural tears • Autologous serum (from the patient’s own peripheral blood) is the first introduced and most commonly used product. Despite several RCT showed its safety and efficacy, a recent Cochraine meta-analysis failed to show significant results due to low evidence. • Homologous serum sources including allogeneic serum obtained from healthy donors, and umbilical cord blood serum collected at the time of delivery, are efficient alternatives, especially when autologous serum therapy is C/I or not appropriate. • Platelet-derived eye drops are prepared and used in various standardized preparations like platelet-rich plasma, plasma rich in growth factors, and platelet lysate. • Future perspectives include the introduction of tailored eye drops, screened for the proper content of growth factors and cytokines according to each patient and ocular surface disease.
CONCLUSIONS • Diagnosis of peripheral ulcerative keratitis requires thorough history and physical examinations given the numerous causes. • Prompt treatment including immunosuppressive medication and sometimes antimicrobial medication is crucial to preventing serious visual consequences including corneal perforation and blindness
References: 1. Cornea- fundamentals, diagnosis and management by Krachmer 2. Myron Yanoff and Jay S. Duker 3. Post-graduate ophthalmology by Zia Chaudhari 4. Corneal disorder by Leibowitz 5. External Diseases and Cornea –AAO Series

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Peripheral Ulcerative Keratits

  • 1.
  • 2.
    A group of Destructiveinflammatory diseases involving the Peripheral cornea Final common pathway  sloughing of corneal epithelium and keratolysis (corneal melting) • Begins with crescentic destructive inflammation at corneal periphery • Ass w/ epithelial defects, stromal inflammatory cells, Progressive stromal melting , degradation and necrosis perforation • May be associated with Episcleritis, Necrotizing Scleritis, Iridocyclitis
  • 3.
    PERIPHERAL CORNEA • Noclear-cut borders delineating peripheral cornea • Portion located b/w central 50% of cornea and limbus. • An arbitrary central limit beginning at 3.5–4.5 mm from the visual axis extending to junction of ill-defined transition b/w limbus and conjunctiva. • Directly adjacent to limbus and internal angle structures • Highly vascular zone with associated lymphatic tissue, scleral collagen, corneal collagen and limbal stem cells. • Thus, vascular inflammatory disorders, limbal infections, collagen vascular disorders, neoplastic disease, and local degenerations may affect peripheral cornea in a distinctive way
  • 4.
    Distinct anatomic differencesof the peripheral cornea • Thickest region of the cornea (up to 0.7 mm) with tight collagen bundle packing • A vascular arcade originating from the anterior ciliary arteries extends approx. 0.5 mm into the clear cornea • Unlike central avascular cornea , peripheral cornea derives part of its blood supply from Ant conjunctiva and deep episcleral vessels which are a source of immunocompetent cells • The accompanying lymphatics, which drain to the regional lymph nodes.
  • 5.
     The adjacentconjunctival blood vessels and lymphatics provide the peripheral cornea • Access to afferent and efferent arc of the immune system • Source of inflammatory effector cells and cytokines involved in the production of collagenase and proteoglycanase, which contribute to corneal degradation.  Unique immunologic characteristics of the peripheral cornea • presence of more Langerhans’ cells, • higher concentrations of IgM and C1 (important in the activation of the classic complement pathway), due to diffusion from limbal vessels due to large size
  • 6.
    ETIOLOGY  Majority- localand systemic immunological processes  Few cases - infectious Ocular Non-infectious Infectious • Idiopathic • Acne rosacea (ocular) • Mooren’s ulcer • Traumatic, post surgical • Exposure/neuroparalytic keratopathy • Marginal keratitis • Contact lens use • Nutritional deficiencies • Bacterial (Staphylococcus, Streptococcus, Gonococcus [rare] • Viral (HSV,HZV) • Amoebic (Acanthamoeba) • Fungal
  • 7.
    Systemic Noninfectious Infectious Collagen vascular diseases/vasculitis •Rheumatoid arthritis • ANCA-associated vasculitides • Wegener’s granulomatosis • Polyarteritis nodosa • Microscopic polyangiitis • Churg–Strauss syndrome • Relapsing polychondritis • SLE • Progressive systemic sclerosis or scleroderma • Giant cell arteritis Other systemic autoimmune • Sjögren’s syndrome • Cicatricial pemphigoid (especially with trichiasis) • Inflammatory bowel disease • Sarcoidosis • Leukemia/malignancy • Giant cell arteritis • Gonorrhea • Bacillary dysentery- Salmonella, Shigella • Tuberculosis • Syphilis • Varicella zoster • Helminthiasis • Hep C • HIV • Borreliosis(Lyme disease )
  • 8.
    PATHOGENESIS Exact pathophysiologic mechanisms- unclear. PROPOSED MECHANISMS IN IMMUNOLOGICAL PATHOGENESIS  Circulating immune complex deposition  Autoimmune reactions to corneal antigens  Hypersensitivity reactions to exogenous antigens Evidence suggests that both humoral and cell-mediated mechanisms (T cell and B cell) are involved.
  • 9.
    Various Etiologies Inflammatory reactionat corneal periphery Immune complex deposition and Complement Activation (C1) Immune vasculitis (damage to the vessel wall ) Further increase in vascular permeability causing leakage Generates chemotactic factors for neutrophils,pro inflammatory cytokines (C3a, C5a) Neutrophils recruited at peripheral cornea liberate proteolytic and collagenolytic enzymes Metalloproteinases production by local resident cells Destruction of corneal stroma
  • 10.
    INFECTIOUS PATHOGENESIS Microorganisms produceproteases  disrupt the extracellular matrix Local productions of cytokines and chemokines diapedesis and migration of neutrophils into peripheral cornea from limbal vessels Cornea inflammation Enzymes released by neutrophils and corneal matrix metalloproteinases activation exacerbate inflammatory necrosis corneal perforation Antimicrobial control of bacterial replication wound healing processes NV vs scarring
  • 11.
    Superinfection • Sta. aureus-Adhesins bind to collagen and other components of exposed Bowman and stroma. • Pseudomonas aeruginosa can bind to molecular receptors exposed on injured epithelial cells. Signs of resolution of active PUK  Resolution of the conjunctival inflammation  Epithelialization of the corneal surface with residual corneal thinning and a vascularized scar.
  • 12.
    OCULAR MANIFESTATIONS Presenting symptomsof PUK -not specific. Foreign body sensation, watering, pain, and photophobia - epithelial erosion and ulceration. Pain is severe - associated scleritis Pain without scleral involvement- Mooren’s Ulcer Decreased Vision - inflammatory process proceeds centrally. • Photophobia with reduced visual acuity - Associated anterior uveitis
  • 13.
    Slit lamp Examination •Crescent shaped destructive lesion at juxta limbal corneal stroma • W/in 2mm of limbus ,accompanied by varying degrees of vaso-occlusion of adjacent limbal networks • Epithelial defect • Stromal thinning w/ yellow white infiltrates –inflammatory cells • In early ds., patchy epithelial involvement;stroma –near normal thickness • Varying degrees of corneal thinning Thinning due to keratolysis (corneal melting), may occur at any clock hour of the peripheral cornea, with or w/out an intervening clear zone from limbus
  • 15.
    • Disease processmay progress circumferentially, centrally, and posteriorly  perforation. • Corneal melting may progress very rapidly • Amount of stromal loss underappreciated due to debris and necrotic material deposited at the base of the ulcer. • Becomes more apparent after scrapings
  • 16.
    • Frequently limbal,conjunctival, and episcleral injection occurs. • Concurrent scleritis denotes a higher likelihood of active vasculitis • When PUK is ass w/ scleritis there exists worse ocular and systemic outcome than when there is scleritis alone. • Complications include Severe Astigmatism, Secondary bacterial infection , perforation, cataract ,glaucoma • Cataract and glaucoma may occur as a result of the inflammatory process or the use of corticosteroids
  • 17.
    SYSTEMIC ASSOCIATIONS 50% ofpatients with PUK may have an associated systemic disease with the large majority being the collagen vascular diseases (CVDs). Rheumatoid arthritis (RA) is most common CVD ass w/ PUK, likely due to its high prevalence in the population (affecting 2.5–3% of adults). Wegener’s granulomatosis and other ANCA-associated vasculitides, although relatively rare, are an important cause of PUK.
  • 18.
    • Generally, patientswith RA-associated PUK have an established advanced systemic diagnosis • However, in up to 25% patients, PUK may be the initial presenting feature of a potentially lethal undiagnosed systemic vasculitis, highlighting the importance of a thorough systemic evaluation.
  • 19.
    General Examination 0fThe Head And Extremities In Peripheral Ulcerative Keratitis Clinical Finding Associated Systemic Diseases Saddle Nose Deformity Relapsing Polychondritis, WG Auricular Pinnae Deformity Relapsing Polychondritis Nasal Mucosal Ulcers WG Oral/Lip/Tongue Mucosal Ulcers SLE, Sjogrens Syndrome Facial "Butterfly" Rash SLE Alopecia SLE, Progressive Systemic Sclerosis, Relapsing Polychondritis Hypo/Hyperpigmentation (Scalp, Face) PSS
  • 20.
    Clinical Finding AssociatedSystemic Diseases Loss Of Facial Expression Rosacea, PSS Facial Telangiectasias Rosacea Rhinophyma All Vasculitic Syndromes Facial/Arms/Legs Rashes, Ulcers PSS Facial/Arms/Legs Taught Skin GCA Temporal Artery Erythema/Tenderness PSS, SLE, GCA, Sjogrens Syndrome Raynaud’s Phenomenon (Fingers) All Vasulitic Syndromes Ulcers In Fingertips RA, SLE, WG, Churg Strauss, PAN Subcutaneous Nodules In Arms And Legs All Vasculitic Syndromes
  • 21.
    DIAGNOSTIC WORK-UP • Athorough ocular history, contact lens wear, current and previous medication for any diseases, trauma, or surgery • A comprehensive Systemic review to r/o systemic diseases ass w/ PUK • A complete ophthalmic examination to exclude local pathologies is critical.
  • 22.
    Tests to excludeinfectious etiologies Corneal scrapings and subject to 1. Gram Stain , KOH 2. Culture 3. Limulus Lysate Assay  Rapid and reliable method  Detect minute amounts of Gram Negative endotoxin  Prompt selection of appropriate anti microbial
  • 23.
    LABORATORY INVESTIGATIONS 1. CBC, ESR 2. RF( +ve in 80 % patients with RA) 3. Angiotensin Converting Enzyme ( Sarcoidosis) 4. Anti Nuclear Antibody(ANA) (SLE ,RA) 5. Antibody to Double Strand DNA (anti –ds DNA)(SLE) 6. Antibody to small nuclear ribonucleoprotein (anti-RNP )(SLE) 7. Anti neutrophil cytoplasmic antibodies (ANCA ) c-ANCA in WG 8. Anti CCP 9. Urine Analysis 10. HBsAg 11. FTA –ABS 12. CXR 13. Sinus CT
  • 24.
    14 . Biopsyof the bulbar conjunctiva adjacent to the ulcerating cornea should be considered, especially in suspected autoimmune associated PUK. Available modalities for studying the specimens include Light and electron microscopy Immunoprobes Polymerase chain reaction (PCR)
  • 25.
    LABORATORY TESTS FORSUSPECTED SYSTEMIC DISEASES IN PUK Systemic Diseases Laboratory Tests Systemic lupus erythematosus ESR, ANA (anti-dsDNA, anti-Sm) IgG, Complement, Cryogl Rheumatoid arthritis ESR, RF, ANA(anti-RNP), CIC, Complement, Cryog, joint X-rays Progressive systemic sclerosis ESR, ANA (anti-centromere, anti-Scl-70), RF, IgG, CIC Sjoren’s syndrome ESR, ANA (anti-RO, anti-La), CIC, RF, Cryo, IgG, IgA, IgM, sialography
  • 26.
    Systemic Diseases LaboratoryTests Polyarteritis nodosa ESR, HbSAg, IgG, Cryog,complement, CIC, angiography Churg-Strauss ESR, WBC/Eosinophil count, IgE, CIC, Chest X-ray Relapsing polychondritis CIC, Complement Wegener’s granulomatosis ESR, IgA, IgE, RF, ANCA, CIC, sinus and chest x-ray, BUN, creat Giant cell arteritis ESR, CRP, CIC, IgG
  • 27.
    • Infectious causesneed to be excluded by the appropriate culture techniques • If infections are suspected- Antimicrobial therapy administered • Prophylactic topical antibiotics - prevent secondary infections ENHANCED LUBRICATION • Dilutes effect of inflammatory cytokines in preocular tear film • Many rheumatoid patients have KCS as a manifest of 2◦ Sjogren syndrome Melting stops or slows down if epithelium made to heal by Lubricants, Pathching, Bandage Contact lens Medical Treatment
  • 28.
    Topical Collagenase Inhibitors •Sodium citrate 10 % • Acetylcysteine solution 20 % • Medroxy progesterone 1% Systemic Collagenase inhibitors • Tetracycline
  • 29.
    Topical corticosteroids • consideredas initial therapy in milder, u/l cases (typically when not associated with a systemic CVD) • Useful in mild cases of RA-associated PUK • May be harmful in a subset of vasculitic PUK because they inhibit new collagen production • Not effective in WG, microscopic polyangiitis, Churg–Strauss syndrome, and PAN • In these cases, corticosteroids may promote progression and even enhance perforation and thus must be used judiciously.
  • 30.
    Systemic corticosteroids • Oralprednisone 1 mg/kg/day- very commonly used for the a/c management of more severe cases of PUK. • If there is progression, pulsed methylprednisolone (0.5-1.0 g) for 3 consecutive days may be effective. • Corticosteroids alone may be inadequate to control the progressive ocular disease process • Prolonged use of corticosteroids - significant systemic side-effects necessitating alternative steroid-sparing immunosuppressive agents.
  • 31.
    Systemic immunosuppressants • Immunomodulatoryagents, such as methotrexate, azathioprine, or cyclosporin A, mycophenolate mofetyl and Cytotoxic agents like cyclophosphamide have demonstrated clinical efficacy for inflammatory eye disease Indications for IMMUNOSUPPRESSION in PUK 1. ass w/ potentially lethal systemic vasculitic syndromes such as PAN, RA, SLE, PSS, Sjogren’s syndrome,WG, allergic angiitis of Churg-Strauss,GCA 2. ass w/ necrotizing scleritis and vasculitis confirmed by histopathologic analysis of ocular tissue. 3. B/l and /or progressive Mooren’s ulcer. 4. unresponsive to aggressive conventional medical and surgical therapy
  • 32.
    • Studies suggestthat cyclosporin A with dosages in the range of 2.5 mg/kg/day may be a reasonable initial choice for In idiopathic PUK, (especially if nephrotoxicity is not a concern) • In cases of PUK ass w/ WG , necrotizing scleritis or Severe or rapidly progressive PUK associated with other CVDs First-line therapy- Cytotoxic immunosuppressives, such as cyclophosphamide (2 mg/kg/day), together with corticosteroids (oral or pulse intravenous) Maintenance- oral or SC methotrexate(7.5 -12.5 mg /week)
  • 33.
    Drug MOA DiseaseDosage SIDE EFFECTS MONITOR Azathioprine Prodrug: 6 Mercaptopurine ,Purine nucleotide analog Blocks RNA ,DNA &protein synthesis, S phase specific Rheumatoid arthritis, WG, and Relapsing polychondritis (2nd Choice) 1-3 mg/kg/day ORAL Bone marrow suppression (reversible), increased risk of malignancies( NHL) LFT,Absolute platelet count Cyclophospha mide Alkylating agent , DNA to DNA cross linking , DNA miscoding WG and PAN (1st Choice), Rheumatoid arthritis 2 mg/kg/day ORAL/IM Bone Marrow Depression Blood Counts,LFT Methotrexate Inhibits DHFR, Bloocks DNA &RNA synthesis &repair , S phase specific Rheumatoid arthritis (1st choice), Maintenance for WG 7.5-12.5 mg/wk p.o. or IM Hepatotoxicity, cytopenia,int erstitial pneumonia CBC, LFT every 2 weeks for 1st month ,then every 4- 6 weeks, RFT Cyclosporine Inhibits IL 2 and othe rproinflammator y cytokines by inhibiting NF –AT transcription factor for signalling Idiopathic (1st Choice), Rheumatoid arthritis, Relapsing polychondritis (2nd Choice) 2–2.5 mg/kg Twice Daily Transient increase in creatinine (50 ),gingival hyperplasia (40 %),hirsuitism, nephrotoxicit y, HTN RFT, LFT, BP every 2 weeks Mycophenolate mofetil Selectove inhibitor of IMP Dehydrogenae , inhibits de novo synthesis , decreasing DNA synthesis Alternative to Azathioprine 1 g Twice Daily, with Max of 1.5 g Twice Daily Hepatotoxicity , leukopenia CBC, LFT
  • 34.
    Biological agents • Theefficacy of Biologic agents such as the anti-tissue necrosis factor (TNF) and anti-B cell monoclonal antibodies in the treatment of RA, WG, spondylo-arthropathies, inflammatory bowel disease as well as other systemic vasculitis suggest that they have a role. • Systemic therapy should be continued for a period of 6 months to 1 year after initial control of inflammation has been achieved
  • 35.
    Drug Disease Dosage Biologicagents Anti-TNF Inflixamab RA, Crohn’s disease RA: 3 mg/kg IV infusion at 0, 2, 6 wk then every 8 wk. Crohn’s diseases 5 mg/kg 0, 2 and 6 wk then every 8 wk. Dosage may be increased interval of treatment reduced Adalimumab Wegener’s granulomatosis, RA 40 mg s.c. every 4 wk Anti CD22 (B cell) Rituximab WG: 375 mg/m2 once weekly for 4 wk. RA: Two 1000 mg IV infusion separated by 2 wk, preceeded by 30 min with 100 mg of IV prednisolone Dosage maybe increased interval of treatment reduced
  • 36.
    Surgical Treatment Surgical treatmentsmainly to maintain the integrity of the globe and are typically palliative. Conjunctival resection • Temporarily remove the limbal source of local cellular mediators and collagenases important in progression of the disease process • May be of great diagnostic help Patch graft • When a perforation is too large for tissue adhesive to seal the leak, some type of patch graft will be necessary. • This may range from a small tapered plug of corneal tissue to a penetrating keratoplasty Tissue adhesive Application of Isobutyl cyanoacrylate glue , forms a biological barrier b/w host cornea and the reepithelializing conjunctiva and the immune
  • 37.
    Superficial lamellar keratectomy •arrest the inflammatory process and allow healing Rehabilitative surgical therapy • Initial lamellar tectonic grafting followed by central penetrating keratoplasty • In advanced cases • LKP is the most widely practiced surgery – in 2 stages • For an ulcer < 1/2 circle of limbus and the central 7-8 mm of cornea uninvolved, crescent shaped lamellar graft can be used. • For an ulcer > 2/3 of a circle of limbus where the central 7-8 mm of cornea is intact, a doughnut shaped lamellar graft is recommended.
  • 38.
    1st stage • Lamellarflap that encloses corneal pathology –microkeratome • Flap lifted to ensure underlying stroma clear, replaced 4-6 week interval • Flap stabilizes and partially adhere 2nd stage • Trephine over previously made flap to a depth slightly below initial lamellar dissection • 1mm flap rim left behind, Flap removed • Donor tissue transferred to host bed  Spontaneously adhere  10-0’nylon sutures  Bandage Contact Lens Femto second laser can be used for making the flap
  • 39.
    • Amniotic Membranetransplantation- preserve the integrity of the globe • Cyanoacrylate adhesive in conjunction with immunosuppressive therapy may be used in patients with impending perforation and may delay the need for tectonic corneal surgery. • Conjunctival flaps  control corneal melting in difficult to manage microbial keratitis,  but best avoided in immune mediated disease  Bringing conjunctival vasculature closer to area of corneal disease accelerate melting
  • 40.
    COURSE AND OUTCOME •The course, duration, and outcome are variable and dependent on the underlying cause of the PUK and on prompt and appropriate management. • Many patients with mild or moderate PUK may maintain good vision if the inflammatory process is rapidly controlled. • The prognosis is more guarded when PUK is ass with a systemic CVD. • Significant visual loss and ocular morbidity may develop with corneal perforation. • Both ocular and systemic prognosis is more guarded when there is concomitant scleritis, especially necrotizing scleritis
  • 41.
    Mooren’s ulcer • strictlya PUK, with no associated scleritis. • Bowman published the first report of Mooren’s ulcer in 1849. • In 1854, McKenzie described it as ‘c/c serpiginous ulcer of the cornea or ulcus roden.’ • The disorder was named as Mooren’s ulcer after Dr Mooren, who was the first to clearly describe this insidious corneal problem and define it as a clinical entity
  • 42.
    • Mooren’s ulcercan occur in all age groups, but the vast majority present b/w 40 and 70 years. • Disease can occur in either sex, but men outnumber women. • U/l involvement is more common • Interpalpabral limbus is involved most often, followed by the inferior and then the superior limbus. • occurs in complete absence of any diagnosable systemic disorder that could be responsible for progressive destruction of the cornea.
  • 43.
    • rare, degenerative,superficial ,idiopathic disease • painful, rapidly progressive, chronic ulcerative keratitis that begin peripherally and progresses circumferentially , then centripetally • single or multi-centric crescent shaped peripheral corneal ulcer with a characteristic steep, infiltrated, undermined, or overhanging edges
  • 44.
    Healing: Corneal epithelializationand vascularization associated with scarring and thinning. Advanced cases: most of the cornea is lost leaving behind a central island surrounded by area of grossly thinned, scarred, and vascularized tissue.Corneal perforation is uncommon.
  • 45.
    Epidemiology Wood and Kaufmandescribed two clinical types of Mooren’s ulcer. Limited type(typical or benign Mooren’s ulcer) • U/l with mild to moderate symptoms, • generally responds well to medical and surgical treatment. • occur in older patients Atypical or malignant Mooren’s ulcer • B/l although both eyes may not be affected simultaneously • relatively more pain and generally a poor response to therapy. • younger patients • progresses relentlessly and is more likely to result in corneal perforation.
  • 46.
    Watson, based onclinical presentation and anterior segment fluorescein angiographic findings, divided Mooren’s ulcer into three distinct varieties. U/l Mooren’s ulceration • painful progressive corneal ulceration in elderly • Ass w/ nonperfusion of the superficial vascular plexus of the ant segment B/l aggressive Mooren’s ulceration • occurs in young patients • progresses circumferentially, then centrally in the cornea. • Vascular leakage and new vessel formation, extending into base of ulcer. B/l indolent Mooren’s ulceration • usually in middle-aged patients • presenting with progressive peripheral corneal guttering in both eyes, with little inflammatory response
  • 48.
    Pathogenesis • Although etiologyunknown, precipitating factors include accidental trauma or surgery and exposure to parasitic infection(incidence is higher in areas of endemic infection) • Ass w/ infections- helminthiasis, hep C, herpes simplex and zoster. • Characteristics of an autoimmune process • decrease in number of suppressor T cells relative to no of helper T cells • elevated IgA levels • Higher circulating IgG antibodies and immune complexes to human corneal and conjunctival epithelium. • Ratio of CD4/CD8 cells and B7-2/antigen-presenting cells higher • Ass w/ HLA-DR17
  • 49.
    • Antibodies toan auto-antigen that exists in corneal stroma. • The antigen, known as ‘cornea-associated antigen’, has an amino acid sequence identical to that of calgranulin C of neutrophils. • No change from normal vascular architecture except an extension of new vessels into the ulcer. • Trauma or infection alter normal corneal antigenautoimmune response • Altered antigen taken up by macrophages(APC),  T-cell activation, differentiation, and proliferation. • Lymphocytes return to the conjunctival vesselsocular inflammation.
  • 50.
    • Corneal edemafrom inflammation  easier diffusion of immune components into pericentral and central cornea from the limbal vessels • Thus cornea is damaged, liberating more altered corneal antigens that aggravate and perpetuate the process until the corneal stroma is completely destroyed • Molecular mimicry may be involved, stimulating an auto immune response. • Deposition of immune complex over peripheral cornea • Auto immune lysis of epithelium with release of collagenolytic enzymes.
  • 51.
    • Grey infiltrates, which break down, forming small ulcers that spread and coalesce. • It destroys the epithelium and superficial stromal lamellae, forming a whitish overhanging edge, while the base quickly becomes vascularised.
  • 52.
    SYMPTOMS • severe andpersistent neuralgic pain. • Redness Photophobia Lacrimation • Decrease in visual acuity due to associated iritis, central corneal involvement and irregular astigmatism.
  • 53.
    • The followingstepwise approach to management is recommended 1.Topical steroids and antibiotics-Unilateral, mild-moderate cases 2. Conjunctival resection Conjunctiva is resected up to 2 clock hours on either side to bare sclera and extend 3–4 mm from the limbus. Excision of a 4-7 mm strip of adjacent conjunctiva may prove successful by eliminating conjunctival sources of collagenase , proteoglycanase and other inflammatory mediators. 3. Topical acetylcysteine 10% and L-cysteine (0 .2 molar), topical cyclosporine
  • 54.
    4. In caseof Perforation: • ulcer debridement with cyanoacrylate adhesive and soft contact lenses. • Lamellar keratoplasty with IV methotrexate– halt the process 5.Topical IF 2 alpha, Infliximab 6. Systemic immunosuppression-Bilateral or progressive Mooren's ulcer that fail w/therapeutic steroids and conjunctival resection will require systemic cytotoxic chemotherapy 5.Additional surgery: Keratoepithelioplasty Lamellar keratectomy
  • 55.
    • A stepwiseapproach to treatment of Mooren's ulcer is used. • Treatment proceeds from topical corticosteroids to conjunctival excision with or without lamellar keratectomy and, finally, to systemic immunosuppression. • However, if a patient presents with b/l simultaneous ulcer or advanced disease or progresses rapidly, little time is spent waiting for a therapeutic response to topical therapy, and treatment consisting of conjunctival excision with systemic immunosuppression is undertaken.
  • 56.
    ROSACEA KERATITIS(acne rosacea) •c/c acneiform inflammatory disorder chiefly affecting skin and eyes • unknown etiology but may be related to overexpression of cathelicidin antimicrobial peptides. • Cathelicidins cause neutrophil recruitment, angiogenesis, and cytokine release, which may contribute to the inflammatory skin changes • sebaceous gland dysfunction of the face, neck, and shoulders • More common in women in 4th decade • More severe manifestations occur in men • Alcohol can contribute to a worsening of this disorder because of its effect on vasomotor stability
  • 57.
    CLINICAL PRESENTATION • Morecommon in fair-skinned individuals, it may simply be more difficult to diagnose in people with dark skin. • Skin manifestations precede Corneal manifestations except in 10% of cases • Facial lesions consist of telangiectasias, recurrent papules and pustules, and midfacial erythema • Characteristic malar rash with unpredictable flushing episodes, ass w/ consumption of alcohol, coffee, or other foods • Hypertrophic cutaneous changes occur late in the disease process-Rhinophyma
  • 58.
    • Excessive sebumsecretion with frequently recalcitrant c/c blepharitis. • Eyelid margin telangiectasia • Meibomian gland distortion, disruption, and dysfunction, which can lead to recurrent chalazia. • c/c conjunctivitis, marginal corneal infiltrates ,sterile ulceration, episcleritis, or iridocyclitis. • If properly treated, these lesions can resolve with few sequelae. • Repeated bouts of ocular surface inflammation can cause corneal neovascularization and scarring
  • 59.
    CORNEA  Affects inferiorquadrant of cornea  Punctate epithelial erosions  Vascular invasion of the peripheral cornea with subepithelial infiltrates  Progresses centrally and circumferentially –ulceration ,scarring and perforation • Limbal involvement – Nodular conjunctivits, Nodular scleritis
  • 61.
    MANAGEMENT • Systemic tetracyclinesas the mainstay of therapy. • Tetracyclines have anti-inflammatory properties that include suppression of leukocyte migration, reduced production of NO and ROS, inhibition of MMPs, and inhibition of phospholipase A2 and reduce irritative FFA and diglycerides by suppressing bacteriallipases. • With time, oral therapy with doxycycline or minocycline can be tapered. • Oral tetracycline 250 mg QID for 6 – 8 weeks • After that dosage to be tapered by 250 mg per week • Tetracycline 250mg OD for a year before discontinuing
  • 62.
    • Topical metronidazole0.75% gel, metronidazole 1% cream, or azelaic acid gel 15% to the affected facial areas reduce facial erythema. • PUK can be ass w/ infectious agents in rosacea, or it may have a sterile inflammatory etiology. • Once it is ascertained that PUK is noninfectious, topical corticosteroids, used in reducing sterile inflammation and enhancing epithelialization of the cornea. • In advanced cases with scarring and neovascularization, conservative therapy is generally recommended. • Penetrating keratoplasty is a high-risk procedure in rosacea patients; it may result in a poor prognosis if the ocular surface is severely compromised. • Light-pulse treatment may help reduce eyelid erythema.
  • 63.
    RHEUMATOID ARTHRITIS • Commonestsystemic association • Involves both eyes in 30% cases • Tends to affect during late and advanced vasculitic phase • Ass w/ all types of anterior scleritis including Diffuse, nodular, necrotizing scleritis with inflammation and necrotizing scleritis without inflammation (scleromalacia perforans), as well as posterior scleritis
  • 64.
    • Corneal findings PUK  Sclerosing keratitis(gradual thickening and opacification of corneal stroma adjacent to site of scleritis)  Acute stromal keratitis  Limbal guttering  Keratolysis  Keratoconjunctivitis sicca
  • 65.
    PATHOGENESIS • Immune complexdeposition in peripheral cornea and limbal vessels • Chemotaxis of inflammatory cells There are 2 types of PUK in RA 1)Paracentral 2)Peripheral Paracentral keratolysis • In patients with severe dry eye • Mechanism – Altered epitheliumInflammatory mediators enter the stromaStimulate a sterile keratolysis and a T cell infiltrate • Progresses both centrally and circumferentially • Unresponsive to topical therapy
  • 66.
    2.Peripheral keratolysis • Thereis an obliterative Microangiitis at the level of limbal vascular arcades • Deposition of Immune complexes in limbal vessels • Causes an immune microangiopathy and obliterative microangiitis • leakage of inflammatory cells and proteins • collagenases and proteases are released by neutrophils • corneal melting
  • 68.
    Rheumatoid polyarthritis suspectedin an HIV patient with scleritis ,peripheral ulcerative keratitis, and anterior uveitis by Domngang Noche C1, Singwé-Ngandeu M2, Bella AL2 • 37/F • K/c/o AIDS on ART for 3 years • H/o c/c polyarthropathies x past 6 months • Presented with RE- nodular scleritis LE-PUK,necrotizing scleritis,granulomatous ant uveitis • Ophthalmic workup- negative for opportunistic infections and potential causes of scleritis and PUK • Patient unresponsive to topical antibacterial and anti-inflammatory treatment
  • 69.
    • Sulfasalazine started •Ocular lesion resolution and articular swelling improvement observed in <6 weeks • Based on American College of Rheumatology classification criteria, and considering the good response to the treatment (sulfasalazine), diagnosis of Rheumatoid Arthritis with ocular involvement was made in the absence of confirmatory lab tests result.
  • 70.
    Necrotising Scleritis andPeripheral Ulcerative Keratitis Associated with Rheumatoid Arthritis Treated with Rituximab. By Hardy S1, Hashemi K1, Catanese M1, Candil M2, Zufferey P2, Gabison E1, Guex-Crosier Y1. • 3 cases of PUK ass/ w/ RA • 2 patients - necrotising scleritis • In the 2 patients who received the induction therapy with rituximab , good initial therapeutic response observed - stabilized from an ocular standpoint as compared to patient on only high dose corticosteroid • The patient who received only corticosteroid developed massive colon perforation as well as acute renal insufficiency early on in the Rx course • In patients receiving rituximab, eventually the drug had to be switched to other molecules, either due to other systemic symptoms from the disease itself or due to adverse effect of this treatment. • This study contributes to other reports stating that rituximab can be an effective treatment for refractory ocular complications in RA, at least as an induction therapy.
  • 71.
    WEGENER’S GRANULOMATOSIS(Granulomatosis withPolyangiitis) • Multisystem necrotizing granulomatous vasculitic disease • Common ophthalmic manifestations-Usually bilateral  Orbital involvement with proptosis  PUK  adjacent necrotizing scleritis  Sometimes conjunctivitis or episcleritis PUK • can be the initial clinical manifestation • May be the presenting or only sign of the disease. • Crescentic peripheral corneal ulcer that resembles Mooren’s ulcer • Scleral involvement is invariably present C/F from Moorens
  • 72.
    HISTOPATHOLOGY • Lymphocytes andplasma cells predominate the substantia propria • Sclera and episclera may show a Granulomatous reaction with epithelioid cell and giant cells • Areas of active collagen degradation can be seen p ANCA testing is positive MANAGEMENT Systemic immunosuppression with cyclophosphamide and steroids
  • 73.
    Peripheral Ulcerative KeratitisAssociated with Granulomatosis with Polyangiitis Emerging Despite Cyclophosphamide, Successfully Treated with Rituximab. By Fujita Y1, Fukui S1,2, Endo Y1, Tsuji S1, Takatani A1, Shimizu T1, Umeda M1,3, Nishino A1,4, Koga T1,5, Kawashiri SY1,2, Iwamoto N1, Ichinose K1, Tamai M1, Nakamura H1, Origuchi T1,6, Ueki R7, Uematsu M7, Ishida K8, Abe K8, Kawakami A1. • 67/M • K/c/o GPA (right maxillary sinusitis, proteinase 3 ANCA positivity) • RE scleritis • A conjunctival biopsy specimen -neutrophil-predominant infiltration around the vessels without granuloma • Pulsed methylprednisolone and IV cyclophosphamide pulse therapy started.
  • 74.
    • However, itwas ineffective, and PUK newly emerged. • Promptly switched the patient from IVCY to rituximab, which controlled ocular disease • Amniotic membrane transplantation.
  • 75.
    A case reportof the orbit, ocular association and the lung in granulomatosis with polyangiitis: A diagnostic challenge. By Lu CW1, Liu XF1, Luan Y1, Lu CB2, Zhou DD3, Guo LM1, Sun YB1, Chen SN1, Wu YL1, Hao JL1. • 40/F • Progressive worsening pain, redness and gradual decrease in VA in the BE x 7 years • Previous therapeutic interventions - noncompliant topical and IV dexamethasone x 6 years. • H/o asymptomatic space-occupying lesion in the right middle lung, which was surgically removed 4 months back • 1 month later,h/o surgical removal of Left orbital mass • Diagnosed as PUK ass w/ GPA
  • 76.
    • Corneal lesionswere then treated bilaterally with cryotherapy and oral prednisone and cyclophosphamide were administered. • Following surgery, Condition of the eyes appeared to be stable. • 7 months later, the redness and pain of the right eye recurred, followed by a deep lamellar keratoplasty for the treatment of necrotizing scleritis. • The condition of 2 eyes was subsequently observed to be stable during the 9 month follow-up. • Thus,GPA must be considered in D/d of inflammatory conditions and tumors • Early diagnosis and an appropriate interdisciplinary approach to management, are required to decrease recurrence and morbidity in patients with GPA-mediated inflammatory ocular disease.
  • 77.
    Successful Treatment Strategiesin Granulomatosis With Polyangiitis- Associated Peripheral Ulcerative Keratitis. BYEbrahimiadib N1, Modjtahedi BS, Roohipoor R, Anesi SD, Foster CS. • Patient database of Massachusetts Eye Research and Surgery Institution was searched from 2005 to 2015 to identify patients of PUK w/ GPA. • 16 patients mean follow-up of 64 months duration • Rituximab and cyclophosphamide, either alone or in combination with other agents- most successful agents in controlling inflammation. • Rituximab in 11 patients with remission being achieved in all (100%) • Cyclophosphamide successfully controlled inflammation in 50% (5/10).
  • 78.
    • 2 patients(2/5, 40%) who had achieved initial control on cyclophosphamide had flares of their PUK. • 2 patients (2/11, 18%) patients on rituximab had flares of scleritis and orbital inflammation but not PUK. • 2 patients, 1 in each treatment group, stopped treatment after achieving remission after 6 months of therapy but suffered disease recurrence within 2 months of treatment cessation. • Rituximab achieved a high rate of disease control in PUK patients with GPA and is the preferred agent in halting disease progression.
  • 79.
    Surgical treatment ofperipheral ulcerative keratitis and necrotizing scleritis in granulomatosis with polyangiitis. By Lu CW1, Zhou DD, Wang J, Hao JL • 70/M • progressively worsening pain and redness RE x 6 months, and decreasing VA x 1 month • Was misdiagnosed and treated as keratitis elsewhere, but pain had not decreased. • Diagnosis of necrotizing scleritis with PUK in GPA made • Surgery was performed with necrotic tissue removal, combined with cryotherapy and a conjunctival flap covering surgery. • Symptoms relieved after surgery, and the patient's ocular condition remained stable thereafter. • This case first demonstrates limited ophthalmic GPA (such as, PUK and necrotizing scleritis) that was not aggravated can be successfully treated by combined surgical interventions.
  • 80.
    POLYARTHRITIS NODOSA  PUKmorphologically similar to Mooren’s ulcer with the central overha nging edge may be present.  Usually associated adjacent necrotizing scleritis is present.
  • 81.
    Rapid corneal thinningand perforated ulcerative keratitis in a patient with relapsing polychondritis by Lai THT1, Far N2, Young AL1,2, Jhanji V2,3. • 43 Male • K/c/o relapsing polychondritis w/ b/l scleritis, b/l swelling of pinna, saddle nose and tracheal stenosis. • Right eye pain and redness x 1 month. • O/E- 80% peripheral corneal thinning b/w 3 and 7 o'clock in RE • 1 week of oral corticosteroid use • The degree of corneal thinning worsened to 90% • Subsequently, topical cyclosporine 2% eye drops QID, oral doxycycline 100 mg BD oral vitamin C 2 g OD added • Corneal thinning improved to 60%.
  • 82.
    • Patient rapidlytapered oral prednisolone against medical advice- returned with an a/c drop in vision in RE. • On Slit lamp -peripheral corneal perforation with iris prolapse. • Emergency repair with cyanoacrylate glue. • IV methylprednisolone 1 mg/kg body weight x3 days and 1 g/day and IVIG every 4 weeks. • 3 months postop, a well-formed anterior chamber with glue in situ.
  • 83.
    Gout Keratitis: ACase of Peripheral Ulcerative Keratitis Secondary to Gout With a Review of the Literature. byYazdanyar A1, Rizzuti AE, Mechel E, Denisova K, Lazzaro DR. • 41/ M / k/c/o severe gout disease • Pain and redness of RE • O/E-2 areas of peripheral corneal thinning with overlying epithelial defects. Adjacent to these areas, reflective crystals identified in the corneal stroma. • AS-OCT demonstrated stromal corneal deposits. • Systemic workup was negative aside from an elevated serum uric acid level. • The patient was administered oral prednisone, allopurinol, and colchicine. • 2-month follow-up visit, the patient was asymptomatic and his corneal thinning had significantly improved. • Crystal deposition in the cornea is extremely rare and may be a cause of peripheral ulcerative keratitis.
  • 84.
    Peripheral ulcerative keratitisassociated with chronic malabsorption syndrome and miliary tuberculosis in a child byTarun Arora, Namrata Sharma,1 Adarsh Shashni, and Jeewan S. Titiyal • 16/F • Pain, redness, watering, and blurring of vision RE • O/E RE PUK and nodular scleritis. • Systemic examination-stunted growth, low BMI, enlarged axillary nodes. • Giardia cysts in the stool sample • Histopathology of axillary lymph nodes showed caseating necrosis s/o TB • Diagnosed as PUK with c/c malabsorption syndrome secondary to giardiasis and miliary TB • Oral metronidazole, anti-tubercular treatment, high protein diet and vitamin supplements were started. • Topical steroids were started for peripheral ulcerative lesions following, which the PUK resolved.
  • 85.
    Peripheral Ulcerative KeratitisSecondary to Chronic Hepatitis B Infection. By Wei DW1, Pagnoux C, Chan CC. • 52/Female • RE eye pain and photophobia • Arthralgia, remnants of a maculopapular rash, subsequently facial numbness several weeks later. • BCVA in RE 20/80. • Slit-lamp examination -severe superior corneal thinning without infiltrate. • Corneal ulceration worsened until 10% of the cornea remained. • Laboratory workup was positive for rheumatoid factor and revealed significantly decreased C4 complement, and HBV serology was positive.
  • 86.
    • Clinical history,examinations, and laboratory results suggest HBV-associated cryoglobulinemia and vasculitis. • Management included prednisone, cyclophosphamide, and mycophenolate mofetil for immunosuppression and tenofovir for HBV treatment. • Conjunctival resection and a glue patch were used to reduce inflammation and stabilize corneal melt. • BCVA improved after treatment was initiated. • Two years after initial presentation, her BCVA is 20/30, significantly improved from her vision at presentation.
  • 87.
    Peripheral Ulcerative KeratitisAssociated with HCV-Related Cryoglobulinemia. By Coelho P1, Menezes C2, Gonçalves R1, Rodrigues P1, Seara E1. • 36/M • H/o Hepatitis C and IV illicit drug abuse • moderate pain ,redness and photophobia in RE. • O/E BCVA RE - 20/30 • slight discomfort with eye movement. • Ring shaped peripheral corneal ulcer, with a dense white stromal infiltrate sparing the limbus, plus an adjacent area of mild anterior diffuse nonnecrotizing scleritis. • No intraocular inflammation was present. • Posterior segment was normal.
  • 88.
    • oral prednisolone(1 mg/Kg/day) with gradual tapering. • A dramatic clinical response occurred, with complete resolution of the clinical condition • Systemic analytic workup aiming at autoimmune and infectious diseases was negative, except for high HCV-viral load and the presence of type 2 cryoglobulins.
  • 89.
    Contact lens wear •reduces the amount of O2 available to the cellular components of cornea • Less tear flow under contact lens than which will normally pass over cornea • may increase the temperature of the cornea • the insertion and removal of lenses may produce regions of microtrauma to the corneal surface, limbus, and adjacent conjunctiva. • Soft contact lenses worn overnight,risk of a corneal infection is increased. • Fequently, patients will not change or clean their contact lens storage cases, which can harbor bacteria and other microorganisms. • certain systemic diseases, such as diabetes and other diseases that slow tissue healing, can increasthe risk of ocular infections. • Dry eye and lagophthalmos serve to compromise the ocular surface. When these conditions are present in an individual who also wears contact lenses, the risk of microbial keratitis increases significantly.
  • 90.
    Mooren Ulcer ina Child Wearing Orthokeratology Contact Lenses. BY Kim J1, Kim MK, Wee WR, Oh JY. • 10/M • Referred for progressive peripheral corneal ulcer RE, despite intensive use of fortified antibiotic eye drops. • H/o overnight orthokeratology lenses x 4 months before presentation of corneal ulcer. • No other history of ocular or systemic trauma and disorders. • Microbiological tests of the lesion were negative. • Systemic evaluation showed no sign of rheumatologic disease. • Under a diagnosis of Mooren ulcer, the patient was treated with topical and systemic corticosteroids. • After four weeks of treatment, the patient's symptoms rapidly disappeared, and corneal ulcer was healed. • Vision recovered to normal with the correction of with-the-rule astigmatism.
  • 91.
    Case-Control Pilot Studyof Soft Contact Lens Wearers With Corneal Infiltrative Events and Healthy Controls. By Richdale K1, Lam DY2, Wagner H3, Zimmerman AB4, Kinoshita BT5, Chalmers R6, Sorbara L7, Szczotka-Flynn L8, Govindarajulu U1, Mitchell GL4. • Purpose :To assess risk factors associated with soft contact lens (SCL)- related corneal infiltrative events (CIEs). • Single-visit, case-control study • 30 Cases defined as current SCL wearers w/ a symptomatic CIE. • For each case, 3 age- and sex-matched controls were enrolled(90 controls ) • 13 to 31 years of age • Subjects supplied a recent health history ,completed the Contact Lens Risk Survey , a standardized scripted medical interview,, and underwent an ocular examination. • Microbial culturing of the ocular surface, SCL, and lens storage case was conducted for all cases.
  • 92.
    • CIE diagnosisincluded contact lens-associated red eye, infiltrative keratitis, and contact lens peripheral ulcer. • Subjects with symptomatic CIEs more likely to harbor substantial levels of gram-negative bioburden on the ocular surface and contact lens. • Significant risk factors for developing a CIE were overnight wear of SCLs, use of multipurpose solution, rinsing SCLs with water, lens storage case older than 6 months, previous "red eye" event, use of ocular drops in the past week, and illness during the past week. • A larger sample size is needed to determine relationships between patient-reported behaviors and exposures, microbial bioburden, and CIE development.
  • 93.
    Peripheral Ulcerative Keratitisfollowing Laser in situ Keratomileusis. By Burkholder BM1, Kuo IC1. • 28/M underwent LASIK • h/o glomerulonephropathy, not disclosed prior • Developed severe postop PUK soon after surgery • Within a week of surgery developed diffuse lamellar keratitis (DLK) not contiguous with PUK • Microbiologic evaluation of the flap interface disclosed no organisms, and no epithelial ingrowth was found. • Both PUK and DLK resolved with topical and oral steroid therapy, and the patient's induced refractive error improved over the 12 months following LASIK. • This case demonstrates importance of diligent history taking to exclude autoimmune disorders or atypical postinfectious sequelae prior to proceeding with keratorefractive intervention.
  • 94.
  • 95.
    Infectious Disorders ofthe Peripheral Cornea • As peripheral cornea is adjacent to the rich vascular supply of the limbus, the limbal lymphatic tissue, and inflammatory cells, microbial keratitis maybe thought to be less frequent than in the central cornea. • However, other factors increase the likelihood of a peripheral corneal infection. Bacterial keratitis • occurs in the inf third of the cornea, could be secondary to corneal exposure. • This should especially be considered in an individual who has suffered multiple ocular infections. • Significant pain • Markedly hyperemic bulbar conjunctiva • Quicly progress to perforation –w/in 24-48 hours • May be Ass w/ uveitis- dense flare and cells , hypopyon
  • 96.
    Common organisms • Staureus ,St epidermidis • Streptococcus pneumoniae and other strs • Pseudomonas aeruginosa (soft contact lens wearers) • Enterobacteriaceae(proteus, Enterobacter) • Moraxella spp Uncommon organisms – Neisseria spp – Listeria – Mycobacterium spp – Nocardia spp – Non-spore-forming anaerobes – Corynebacterium spp – Haemophilus influenzea – Chlamydia trachomatis Intact coneal Keratitis • N. gonorrhoeae • N. meningitidis • C. diphtheriae • H. influenzae • Shigella
  • 97.
    Staphylococcal ulcer • Welldefined • Gray-white stromal infiltrate Streptococcal • Infiltrate purulent • Severe AC reaction • Hypopyon formation Pseudomonas • Rapidly progressive • Suppurative • Necrotic infiltrate • Ass w/ a hypopyon and mucopurulent discharge
  • 98.
    Presentations: • Rapid onsetof pain • Decreased vision • Photophobia • Discharge • Eyelid edema Signs: • Conjunctival injection • White stromal infiltrate associated with an overlying epithelial defect and secondary anterior uveitis • Enlargement of stromal infiltration associated with stromal oedema • Severe infiltration and hypopyon formation • Corneal perforation and endophthalmitis in neglected cases
  • 99.
    Investigations Corneal scraping andculture • Blood agar : most bacteria & fungi except Neisseria, Hemophilus & Moraxella • Chocolate agar : Neisseria, Hemophilus, Moraxella • Sabouraud dextrose agar : fungi • Non nutrient agar : Acanthamoeba • Brain Heart Infusion : Streptococci and Meningococus • Löwenstein-Jensen : Mycobacteria and Nocardia Staining • Gram : Bacteria, fungi, Microsporidia • Giemsa : Bacteria, fungi, Acanthamoeba, Microsporidia • Calcofluor white : Acanthamoeba, fungi, Microsporidia • Acid-fast stain : Mycobacterium, Nocardia Conjunctival swabs : Severe cases and corneal scraping is negative Contact lens cases
  • 100.
    • Rx 1.Fortified topical/periocular injection of antibiotics  GRAM POSITIVE (Staphyloccus, Strep. Pneumoniae)-semi synthetic Penicillin, Vancomycin,Cephalosporin,Bacitracin  GRAM NEGATIVE (Pseudomonas aeruginosa)-Gentamicin, Tobramycin, Carbenicillin, Colistin, Polymyxin B Monotherapy; – Fluoroquinolone (Ciprofloxacin 0.3% or ofloxacin 0.3% 2.Mydriatic –Cycloplegic Prevent synechiae due to accompanying Seveere uveitis 3.Anti-Collagenase compounds- decrease corneal melting 4. Lateral, medial, or both types of tarsorrhaphy may be required to protect the ocular surface and prevent subsequent episodes of infectious keratitis.
  • 101.
    • Microbial keratitisfrom syphilis or TB can produce peripheral corneal changes • Syphilis ass w/ a deep corneal inflammation with deep vessels in the peripheral cornea. • TB produces an a/c inflammation that is more commonly u/l and involves peripheral sectoral portions of the anterior cornea.
  • 102.
    Ocular syphilis masqueradingas bilateral peripheral ulcerative keratitis. By Vignesh AP1, Srinivasan R1, Vijitha S1. • 55/M • recurrent pain and redness BE X 2 months. • BE had b/l peripheral crescent-shaped corneal ulcers suggestive of PUK. • The patient was started on topical steroids elsewhere, but lesion was not showing any signs of healing. • A series of investigations performed- positive VDRL and fluorescent treponemal antibody absorption tests. • Started on systemic penicillin, as well as topical steroids. • The response to the treatment was good and the ulcer began to heal.
  • 103.
    Fungal Keratitis • Lesscommon than BK • 5%-10% of corneal infections • May have devastating effects • severe stromal necrosis • may enter AC by penetrating intact Descemet membrane • difficult control because poor penetration of antimycotic agents. • Filamentous fungal : Asprgillus vs Fasarium • Non Filamentous or Yeast : Candida Predisposing factors : c/c ocular surface disease, long-term use of topical steroids,Contact lens wear, systemic immunosuppression, diabetes,vegetable matter
  • 104.
    Presentation Gradual onset ofFB sensation, Photophobia,Blurred vision,Discharge Signs Filamentous keratitis • Greyish-white infiltrate with indistinct feathery margins. • Often surrounded by finger-like satellite stromal infiltrates Candida keratitis •Yellow-white infiltrate • dense suppuration • similar to bacterial keratitis
  • 105.
    Investigations • Staining - Gramand Giemsa staining - Periodic acid-Schiff (PAS) • Culture - Sabouraud dextrose agar - on blood agar or in enrichment media. - contact lenses • Corneal biopsy - is indicated in the absence of clinical improvement after 3–4 days - if no growth develops from scrapings after a week.
  • 106.
    Management • Removal ofthe epithelium • Topical treatment : hourly for 48 hours - Candida : amphotericin B 0.15% or econazole 1%;or natamycin 5%, or fluconazole 2%, or clotrimazole 1% - Filamentous infection : natamycin 5% or econazole 1%; or amphotericin B 0.15% or miconazole 1%. • A broad-spectrum antibiotic • Cycloplegia • Subconjunctival fluconazole • Systemic antifungals lesions are near the limbus, or for suspected endophthalmitis • Superficial keratectomy • Therapeutic keratoplasty
  • 107.
    Herpes simplex andzoster • can develop peripheral corneal ulcers that do not exhibit typical dendrite formation. • Instead, they develop an irregular epithelium or epithelial defect over the affected area, followed by a region of stromal haze that assumes a crescent shape adjacent to the limbus. • The associated limbus is typically hyperemic and the eye is painful. • Herpes simplex and zoster may also produce a sterile or an infected neurotropic ulcer • Therefore, corneal sensation should always be tested in patients who experience little pain in association with a corneal ulcer. • Treatment is directed at healing the ulcer and protecting the ocular surface with a tarsorrhaphy. • A scleral contact lens may protect the corneal surface once the ulcer is healed.
  • 112.
    Actinobacillus actinomycetemcomitans KeratitisAfter Glaucoma Infiltration Surgery: A Clinical Report and Literature Review. BY Hong J1, Xu J, Cao W, Ji J, Sun X • 56/M / Long-standing h/o open-angle glaucoma BE • h/o ocular pain, redness, blurred vision RE x 12 weeks • h/o Glaucoma infiltration surgery RE x 6 months ago. • 3 months postop, developed peripheral corneal stromal opacities ass w/ a white, thin, cystic bleb, conjunctival injection. • These opacities grew despite topical treatment w/ topical tobramycin, levofloxacin, natamycin, amikacin, metronidazole eye drops. • Multiple corneal scrapings revealed no organisms • Aerobic, anaerobic, fungal, or mycobacterial cultures negative
  • 113.
    • RE developeda severe purulent corneal ulcer with a dense hypopyon and required corneal transplantation. • Histopathologic analysis and 16S ribosomal RNA PCR sequencing revealed A actinomycetemcomitans as the causative organism. • Postop, treatment was initiated with topical levofloxacin and cyclosporine, as well as oral levofloxacin and cyclosporine. • Graft and host corneal transparency were maintained at the checkup 1 month after surgery. • Although it is a rare cause of cornealdisease, A actinomycetemcomitans should be suspected in patients with keratitis refractory to topical antibiotic therapy • Delay in diagnosis and appropriate treatment can result in vision loss.
  • 114.
    Differential Diagnosis 1.Marginal keratitisass w/ blepharitis 2.Pellucid marginal degeneration 3. Terrien’s marginal degeneration 4.Furrow degeneration
  • 115.
    Local inflammatory causes •Staphylococcus marginal ulcers, which begin with an infiltrate adjacent to the limbus separated by a characteristic clear zone with progression to ulceration • Rosacea-associated blepharo-conjunctivitis which may result in a peripheral keratitis with or without a clear zone. Non inflammatory cause of thinning of the peripheral cornea Terrien’s marginal degeneration, pellucid marginal degeneration, and marginal furrow can be distinguished from PUK due to lack of inflammation and typically an intact epithelium
  • 116.
    Pellucid marginal cornealdegeneration Unknown etiology  Inferior peripheral corneal thinning and protrusion Point of greatest protrusion is just above the area of maximal thinning Absence of inflammation The involved area is clear, and usually no iron line occurs central to it. Marked against-the-rule astigmatism-Decreased vision Rx-  Contact lenses  Penetrating Keratoplasty  Wedge resection  Lamellar Tectonic grafts  Collagen Cross linking
  • 117.
    Terrien’s degeneration • Uncommonidiopathic thinning and ectasia of superior peripheral cornea • Arcuate lipid deposition and vascularisation in pts aged >40 yrs • 75% Male After 4th decade ,Usually B/l • Initially asymptomatic • Lack of inflammation or epithelial defect • Presence of sup vascularization and advancing linear deposition of lipid • Slow progressive course • No known systemic disorders occur with terrien’s marginal degeneration • Associated with VKC
  • 118.
    • Mild, fineyellow white ,punctate, sub-epithelial or anterior stromal opacities • Peripheral, superficial, fine vascular pannus which start superiorly, spread circumferentially and progresses over years • Linear sub-epithelial opacity at the advancing edge • The thinning slowly begins between the limbus and the line of lipid deposition and is separated from limbus by clear zone • Typically, a steeper sloping of the cornea occurs at the advancing edge. • Gradual vision disturbance due to increasing corneal astigmatism • Pseudopterygia in long standing cases With increased corneal thinning -> ectasia Can perforate after trauma
  • 119.
    HISTOPATHOLOGY • Fibrillar degeneration-Bowman’s Layer and stromal lamellae • Cornea thinner with appearance of a subepithelial pannus Rx • Spectacles • Contact lens-scleral lenses or soft lenses with RGP piggybacking • Tectonic corneal grafting • Topical lubricants and mild topical steroids
  • 121.
    Blood derived eyedrops for the treatment of cornea and ocular surface diseases. By Giannaccare G1, Versura P2, Buzzi M3, Primavera L2, Pellegrini M2, Campos EC2. • Use of blood derived eye drops for the Rx of ocular surface disorders, mainly dry eye disease, persistent corneal epithelial defect, corneal ulcer, ocular surface burn, recurrent corneal erosion and limbal stem-cell deficiency has become increasingly popular in recent years. • MOA -stimulation of cellular proliferation and migration by supplying an active mixture of growth factors and cytokines at the ocular surface, thus mimicking function of the lacking natural tears • Autologous serum (from the patient’s own peripheral blood) is the first introduced and most commonly used product. Despite several RCT showed its safety and efficacy, a recent Cochraine meta-analysis failed to show significant results due to low evidence. • Homologous serum sources including allogeneic serum obtained from healthy donors, and umbilical cord blood serum collected at the time of delivery, are efficient alternatives, especially when autologous serum therapy is C/I or not appropriate. • Platelet-derived eye drops are prepared and used in various standardized preparations like platelet-rich plasma, plasma rich in growth factors, and platelet lysate. • Future perspectives include the introduction of tailored eye drops, screened for the proper content of growth factors and cytokines according to each patient and ocular surface disease.
  • 122.
    CONCLUSIONS • Diagnosis ofperipheral ulcerative keratitis requires thorough history and physical examinations given the numerous causes. • Prompt treatment including immunosuppressive medication and sometimes antimicrobial medication is crucial to preventing serious visual consequences including corneal perforation and blindness
  • 123.
    References: 1. Cornea- fundamentals,diagnosis and management by Krachmer 2. Myron Yanoff and Jay S. Duker 3. Post-graduate ophthalmology by Zia Chaudhari 4. Corneal disorder by Leibowitz 5. External Diseases and Cornea –AAO Series

Editor's Notes

  • #4 contributes to the predilection of this region to be involved in both local and systemic immunologic reactions.
  • #5 contributes to the predilection of this region to be involved in both local and systemic immunologic reactions.
  • #6 the first component of complement
  • #7 (may be a presenting feature of a systemic autoimmune disorder)
  • #9 Mechanisms held responsible are
  • #10 Locally produced or circulating immune complexes lodged in peripheral corneal blood vessels activate the classic complement pathway in the presence of C1 An immune vasculitis damage to the vessel wall with leakage and chemotaxis of various inflammatory cells, proteins, and proinflammatory cytokines
  • #14  associated scleritis,, Corneal infiltrate, and overlying epithelial defect. Typical overhanging edges in –Mooren’s ulcer
  • #15 -Fig. 4-16-2 Progressive circumferential peripheral corneal ulceration. (A) 60-yearold female with idiopathic PUK. Slit-lamp demonstrates depth of stromal necrolysis. There is inflammatory infiltrate at both the peripheral and central edges. (B) Fluorescein staining demonstrates area of ulceration
  • #17 Flourescein Angiography initially shows capillary closure at limbus and then leakage from vascularization extending into base of ulcer
  • #26 anti-dsDNA: antibody to double -stranded DNA CIC: circulating immune complexes C, complement (C3, C4, CH50)
  • #27 anti-dsDNA: antibody to double -stranded DNA CIC: circulating immune complexes C, complement (C3, C4, CH50)
  • #30 (at times combined with conjunctival resection with the latter being not only therapeutic but of potential diagnostic value). Here , Topical cyclosporin A may have some benefit as an adjunct when combined with the appropriate systemic therapy. Topical corticosteroids may be a useful in
  • #32 Azathioprine 2mg/kg/day Cyclosporine 10mg/kg/day????
  • #33 Azathioprine 2mg/kg/day Cyclosporine 10mg/kg/day????
  • #37 CONJUNCTIVAL RESECVTION SUPERFICIAL KERARTECTOMY TISSUE ADHESIVE BCL AMT PATCH GRAFT LAMELLAR KERATOPLASTY PENETRATING KERATOPLASTY KERATOPROSTHESIS
  • #44 Unilateral or bilateral PUK, An early Mooren’s ulcer characterized by a crescent-shaped peripheral ulcer concentric to limbus; the leading edges are undermined and infiltrated.
  • #48 Advanced Mooren’s ulcer characterized by gross destruction of cornea, leaving behind a central island of tissue.
  • #52 Peripheral thinning and an overlying epithelial defect are present in an inflamed eye Peripheral thinning is present in a relatively quiet eye in this patient who had a history of Mooren’s ulcer, now in remission Other causes: Physical trauma, foreign bodies, chemical burns, surgeries such as cataract extraction and keratoplasty
  • #63 Azelaic acid gel 15% is the only gel approved by the US Food and Drug Administration for the treatment of papulopustular rosacea; it is thought to suppress rosacea through anti-inflammatory and antimicrobial mechanisms
  • #64 1- MARGINAL INFILTRATES DIFFUSE ANTERIOR SCLERITIS DIFFUSE NODULAR SCLERITIS
  • #65 STROMAL INFILTRATES
  • #68 PUK with perforation in a patient with RA (B) Corneal/scleral graft for the perforation. Donor was trephined eccentrically to match the limbal involvement.
  • #70 definite RA" is based on the confirmed presence of synovitis in at least 1 joint, absence of an alternative diagnosis that better explains the synovitis, and achievement of a total score of 6 or greater (of a possible 10) from the individual scores in 4 domains: 1.number and site of involved joints (score range 0-5) 2.serologic abnormality (score range 0-3) 3.elevated acute-phase response (score range 0-1) 4.symptom duration (2 levels; range 0-1).
  • #91 MOOREN ulcer can develop in children wearing lens Given rapid progression of Mooren ulcer in a young population, early diagnosis and proper treatment are essential to prevent a devastating outcome.
  • #102 Syphilis 1-month follow-up showing resolution of nodular scleritis and healing ulcerative lesions
  • #109 Herpes simplex virus peripheral keratitis (PCR positive) (A). Image of the same patient with fluorescein (B). Macroulcerative peripheral keratitis in a patient with herpes zoster keratouveitis.
  • #111 Interstitial Keratitis Bacterial infection Parasitic infection Syphilis (could be latent) No skin changes, no Tuberculosis (neg Tb tests) diarrhea, and no contact Leprosy (skin) lens wear Lyme disease (+ hx insect bite with rash) Brucellosis (no fever) Trachoma (no conj scar) Viral infection Systemic disease Herpes simplex virus Cogan's syndr.(hearing okay) Herpes zoster virus (skin) Sarcoidosis (neg. chest XR) Epstein-Barr virus (no symp) Lymphoma (no symptoms) Mumps (no symptoms) Measles (skin) HTLV-1 (no demyelinating)
  • #112 1.Local peripheral ulcer 2.Circumferentoal and central spread Advanced disease Healed stage
  • #117 Pellucid means clear
  • #118 1 peripheral stromal opacification 2 circumferential thinning 3 pseudopterygia other than 3 and 9 o clock position
  • #119 1 peripheral stromal opacification 2 circumferential thinning 3 pseudopterygia other than 3 and 9 o clock position