Peripheral ulcerative keratitis (PUK) is a degenerative condition affecting the peripheral cornea. It is characterized by peripheral corneal thinning and ulceration that spreads circumferentially. PUK can be caused by infection, autoimmune diseases like rheumatoid arthritis, or idiopathic factors. Diagnosis involves examining for signs of peripheral epithelial defects, stromal infiltration, and thinning. Treatment depends on the underlying cause but may include topical antibiotics, steroids, or systemic immunosuppressants to reduce inflammation and prevent worsening of the condition. Prognosis depends on severity and associated systemic diseases, with mild-moderate PUK having a good prognosis generally.

1. PERIPHERAL ULCERATIVE
KERATITIS
DR.PRAKRITI YAGNAM .K

2. - Peripheral corneal ulceration or thinning
- Affects peripheral rather than central
cornea and spreads around the margins
PATHOGENESIS :
Peripheral cornea :
- Arbitrary central limit beginning around
3.5mm. to 4.5mm. from visual axis and
extending to junction of transition between
limbus and sclera or conjunctiva

3. - Anatomically greater thickness (0.7mm.)
- Tight collagen bundle packing
- Vascular arcade originating from anterior
ciliary arteries extend up to 0.5mm into clear
cornea
- Lymphatics drain into regional lymph nodes
INFLAMMATORY MEDIATORS :
- Presence of more Langerhans cells , high
concentration of IgM and C1
- Adjacent conjunctival vessels are source of
inflammatory cells and cytokines

4. - These are involved in production of
collagenase and proteoglycanase which
cause corneal degradation

5. Circulating
immune
complex
deposition
Autoimmune
reaction to
corneal
antigens
Hypersensitivity
reaction to
exogenous
antigens
Activation of
classic
complement
pathway
Immune
vasculitis with
damage to
vessel wall with
leakage
Peripheral corneal
destruction

6. Etiology :

7. 1. Marginal keratitis
2. Moorens ulcer
3. Terrien marginal degeneration
4. Dellen
5. Associated with systemic autoimmune
disease
6. Others – Ocular rosacea
Pellucid marginal degeneration
Furrow degeneration

8. Ocular manifestations – hallmark :
- Epithelial loss demonstrated with
fluorescein
- Stromal inflammatory infiltration
- Thinning caused by keratolysis with or
without clear zone from limbus

9. 1. Marginal keratitis :
- Hypersensitivity reaction against
staphylococcal exotoxins and cell wall
proteins
- May also be caused by Moraxella and
Hemophilus
Antigen from tear film Antibody from vessel
Antigen antibody complexes
associated with lymphocytic infiltration

10. - Lesions are culture negative
- S.aureus is frequently isolated from lid
margins
Symptoms :
- Mild discomfort , redness and lacrimation
- May be bilateral
Signs :
- Chronic blepharitis seen
- Seen commonly at the point of contact of
eyelids with cornea(4,7,10,2’o clock)
- Inferior punctate epitheliopathy

11. - Subepithelial marginal infiltrates separated from
limbus by a clear zone associated with
conjunctival hyperemia
- Epithelial defect smaller than area of infiltrate
- Heals rapidly and recurs rapidly
- Coalescence and circumferential spread
- No AC reaction even with larger infiltrates
Treatment :
- Low dose steroids ( FML/Lotepred) QID for 1-2
weeks with topical antibiotic

12. - Blepharitis is treated
- Recurrence is treated with oral
Tetracycline course
Outcome :
- Resolution occurs in 1-4 weeks
- Residual superficial scarring and slight
thinning with mild pannus
- Iris new vessels may develop but resolves

14. Moorens ulcer :
- Chronic serpiginous or Rodent ulcer
- Rare degenerative autoimmune disease
due to release of collagenolytic enzymes
- Progressive circumferential peripheral
stromal ulceration with later central spread
- Precipitating factors – Corneal insult due to
surgery or infection
- Associated systemic autoimmune disease

15. Less aggressive More aggressive
Affects elderly Young (malignant)
Unilateral Bilateral with severe pain
Responds well Systemic immunosuppression
To medical therapy needed
Pathogenesis : Ischemia due to limbal
vasculitis

16. Symptoms :
- Pain(neuralgia) , photophobia , blurred
vision
Signs :
- Peripheral ulceration involving superficial
1/3 rd stroma with variable epithelial loss
- Ulcer has undermined infiltrated whitish
overhanging leading edge

17. - Vascularization involving bed of ulcer upto
leading edge but not beyond
- Progressive circumferential and central
stromal thinning
- Limbitis present but scleritis rare
- Healing stage – thinning , vascularization
and scarring
- Iritis may occur
- Mainly diagnosis of exclusion

18. Complications :
- Severe astigmatism
- Corneal perforation following minor
trauma(spontaneous rare)
- Secondary bacterial infection
- Cataract
- Glaucoma

19. Treatment :
- Difficult as ischemia is underlying cause
- Topical steroids hourly + Antibiotic
- Topical cyclosporin
- Artificial tears
- Collagenase inhibitors – acetylcysteine
- Systemic immunosuppressants – IV MTX
- If medical treatment not useful then
conjunctival resection with
keratoepithelioplasty done to avoid
collagenases and proteoglycanases

21. TERRIEN MARGINAL DEGENRATION :
- Uncommon idiopathic peripheral thinning
- Associated with scleritis and episcleritis
- 75 % - males are affected and bilateral
Symptoms :
- Usually asymptomatic
- Visual disturbance due to astigmatism
- Sometimes episodic pain and inflammation

22. Signs :
- Fine yellow white (lipid) refractile stromal
opacities with superficial vascularization
(pannus)
- Starts superiorly and spreads overall
- Separated from limbus by a clear zone
- No epithelial defect
- May look like Arcus Senilis

23. Peripheral circumferential thinning
Peripheral gutter
Outer slope shelves gradually and inner
(central)rises sharply with band of lipid
- Perforation rare(spontaneous or traumatic)
- Pseudopterygium may occur

24. Treatment :
- Spectacles
- Contact lenses
- Surgery – Crescentic or annular excision
of gutter with lamellae with full thickness
replacement

26. DELLEN :
- Drying of localized area of cornea due to
pingecula or SCH
- Saucer like thinning with intact epithelium
- Fluorescein pools but does not stain
Treatment : Lubricants and treating cause
Complications : Perforation , descematocele

28. SYSTEMIC AUTOIMMUNE
DISEASES:
•May precede or follow
onset of systemic features
Pathology :
•Immune complex
deposition in peripheral
cornea
•Episcleral and
conjunctival capillary
occlusion
•Secondary cytokine
release and inflammatory
cells recruitment
•Upregulation of
collagenases and
reduced inhibitors

29. - Most common – RA – 30% bilateral
- 2nd most common – Wegener
granulomatosis – ocular signs initial in 50%
- Others – PAN, relapsing polychondritis,SLE
Clinical features :
- Crescentic ulceration with epithelial defect
, thinning and stromal infiltration at limbus
- Extension into sclera seen
- Limbitis,scleritis and episcleritis seen
- No separation between ulcer and limbus

30. - Advanced cases – Contact lens cornea
perforation
Treatment :
- Systemic steroids – Pulse MTX – acute
immunosuppressive-long term
- Topical artificial tears
- Topical antibiotics
- Oral tetracycline
- Topical steroids avoided – may cause
further thinning(relapsing polychondritis
given)

31. - Surgical – conjunctival resection with
keratoepithelioplasty
Rheumatoid paracentral ulcerative keratitis :
- Punched out centrally located lesion with
little infiltrate in a quiet eye
- Perforation occurs rapidly
Treatment :
Topical cyclosporin + Bandage contact lens +
Tissue glue application

32. Corticosteroids usage :
- Mild case of RA – Topical CS
- In GPA,Wegeners,Microscopic
polyangitis,Churg Strauss,PAN topical
corticosteroids may enhance perforation
- So cyclosporin A with systemic therapy used
Systemic steroids :
Acute - Oral prednisolone – 1mg/kg/day
If progression is present – Methyl prednisolone
pulse therapy (0.5 to 1gm.)for 3 days

33. - Severe cases(perforation chance)-
Prednisolone 100mg/day with
immunosuppressants
Immunosuppressants :
- Initial choice – cyclosporin
A(2.5mg/kg/day) if no nephrotoxicity
- GPA associated PUK with necrotizing
scleritis – cyclophosphamide + CS
- Maintenance – Oral /SC MTX

34. Biological agents :
- Anti TNF,Anti B cell monoclonal antibodies
Systemic therapy continued for 6 months to 1
year after subsiding of inflammation

36. OCULAR ROSACEA :
- Common chronic idiopathic dermatosis
- Involves skin exposed to sun – face and
upper neck
- Ocular complications – 6 to 10%
- Facial telangiectasia,papule,pustule
formation and rhinophyma occur

37. Etiology :
- Multifactorial
- Vascular factors + abnormal response to
commensal skin bacteria and Demodex
follicularis mites
- H.pylori exacerbation may also be present
Symptoms :
- Irritation and lacrimation

38. Signs :
Lid – Marginal telangiectasia, posterior
blepharitis, Recurrent meibomian cyst
formation
Conjunctiva – Hyperemia(bulbar),cicatricial
conjunctivitis, conjunctival granuloma and
phlyctenules
Cornea – keratitis at inferior cornea with broad
spade shaped pannus with vessels extending
into stroma with subepithelial infiltrates at
leading edge

39. Treatment :
- Lubricants
- Lid hygiene
- Topical antibiotics ( Azithromycin E/O
QID )
- Steroids for exacerbations
Systemic therapy :
- Tetracyclines(Doxy 100mg. Once daily
for 4 weeks then 50mg.daily)
- Azathioprine for severe disease
- Retinoids may be useful

41. Furrow degeneration :
- Mild peripheral degeneration in adults
- Benign

42. PELLUCID MARGINAL DEGENERATION :
- Type of corneal ectasia
- Progressive peripheral thinning mostly inferior(4
to 8’oclock)
- Bilateral and in adults
Symptoms :
Progressive blurring of vision due to astigmatism

43. Signs :
- Crescenteric 1- 2 mm. band of inferior
corneal thinning 1mm. From limbus
- Epithelium intact – cornea above thinned
area ectatic and flickered
- Myopia and against the rule astigmatism
- Fleischer ring and Vogts striae are rare
- Topography – Butterfly pattern

44. Treatment :
- Spectacles or contact lenses
- Surgical – Large eccentric penetrating
keraropalsty
- Thermocauterisation
- Crescenteric lamellar keratoplasty
- Wedge resection degraded tissue
- Epikeratoplasty
- INTACS
- Collagen cross linking

46. Diagnosis and ancillary testing :
- Previous H/O ocular infection
- Ocular or non ocular herpetic disease
- Contact lens wear
- Medication
- Trauma
- Surgery

47. Workup :
Non infectious – CBC,ESR,CRP,CIC
RF
Anti CCP,c ANCA,p ANCA
Urine analysis
Radiograph
Chest. Sinus

48. Treatment :
Infectious – Antibiotics
Non infectious – Antibiotics to prevent
secondary infection
Prognosis :
Mild to moderate PUK – good
Associated with CVD – guarded
Perforation – Ocular morbidity
Scleritis – guarded both ocular and systemic

49. THANK YOU!!!