Ophthalmology Lectures ; PUK is one of our challenges in ophthalmology ..we must know the cause to ensure good management

1. Peripheral ulcerative keratitis

2. Definition
 Term used to describe a group of destructive
inflammatory diseases involving the peripheral cornea
whose final common pathway is characterized by
sloughing of corneal epithelium and keratolysis.
 Crescent-shaped, juxtalimbal corneal stroma
associated with an epithelial defect, presence of
stromal inflammatory cells, and stromal
degradation.
 Conjunctival, episcleral, and scleral inflammation
are usually evident
 Up to 50% related to systemic disorders
 May progressive circumferentially to involve entire
cornea
 May progress to corneal melting leading to
perforation

3. WHY IN PERIPHERAL CORNEA ?
Peripheral cornea
Unique anatomical & immunological features
The peripheral cornea is adjacent to the vascularized limbus
Close to sclera / episclera / conjunctiva
Unlike the avascular central cornea, the peripheral cornea is
closer to limbal conjunctiva and derives part of its nutrient supply
from the limbal capillary arcade, a source of immunocompetent
cells.
Associated with sub conjunctival lymphaticsafferent arm
↑ IgM in periphery large
↑ Langerhans cells
Reservoir of inflammatory cells
More susceptible to immunological damage

4. What is the Pathogenesis?
The peripheral cornea is adjacent to the
vascularized posterior limbus
Capillary arcades extend 0.5mm into clear cornea
The peripheral cornea and nearby limbus are
unique in their cellular milieu
Peripheral Cornea has more Langerhans cells,
higher concentrations of IgM, and complement
(C1)’

5. .Antigen-presenting cells that express class IIMHC antigens
are capable of mobilization and induction of T cell
responses.
Circulating immune cells, immune complexes,and
complement factors tend to depositadjacent to the
terminal capillary loops of thelimbal vascular arcades
They produce collagenase. Vasculitic processes also cause
damage to vessel walls
This produces a variety of immune phenomena that
manifest in the peripheral cornea

6. Differential diagnosis
Non infectious
Mooren ulcer, Terrien marginal
degeneration, pellucid marginal
degeneration, and furrow
degeneration, marginal keratitis,
blepharitis, contact lens use,
chemical injury to the eyes,
trauma.
infectious
Bacterial
(staph,strept,Gonoco
ccus)
Viral
(HS,HZ)
Amebic(Acanthamoe
ba)
Fungal
OCULAR
Collagene vascular
disease/vasculitis
Other systemic
autoimmune:Sjogrens syndrome
,Sarcoidosis(very
rare),inflammatory bowel disease .
-Malegnancies/leukemia
Gonorrhea,Bacillary
dysentery,T.B,lyme(ver
y
rare),VZ,Helminthiasis
SYSTEMIC

7. History
PUK is frequently a manifestation of an occult systemic disease.
chief complaint/characteristics of present illness/past medical history/ family
history/and a meticulous review of systems.
Ocular symptoms vary, but nonspecific foreign body sensation with or
without eye pain, tearing, photophobia, and reduced visual acuity (secondary
to induced irregular astigmatism), are the most common symptoms for patients
with PUK
.Loss of vision can occur quickly when PUK progresses.
PUK associated with RA, WG, PAN, and RP is often linked with scleritis, and
eye pain may be pronounced in these individuals.
PUK in patients with Mooren ulcer may also produce pain, although there is no
scleral involvement.
Bilateral disease may be present in 21% of patient

8. RA, SLE, PAN, WG, or RP may present with the following symptoms, which
should be emphasized in the review of systems :General -
Constitutional symptoms, such as chills, fever, poor appetite, recent weight
loss, and fatigue
Skin - Rashes, nodules, vesicles, ulcer, nail changes, and periungual infarcts
Respiratory - Coughing, wheezing, pneumonia, and shortness of breath
Cardiac - Chest pain or discomfort and dyspnea
Gastrointestinal - Abdominal pain, nausea, vomiting, difficulty swallowing,
and diarrhea
Musculoskeletal - Muscle or joint pain, arthritis, back pain, and limitation of
motion
Neurologic - Headaches, seizures, psychiatric, paralysis, and
numbness/tingling
Other systemic symptoms - Deafness, swollen ear lobes, ear infections,
)vertigo, and noises in ears (suggestive of RP

9. Ocular&Systemic Examination
Physical Examination should be complete and include an overview of the head
(including the nose, mouth, and external ear), trunk, joints, and extremities. Skin lesions
should also be noted.
Ocular Examination A complete ophthalmic examination should be performed
with special emphasis on the conjunctiva, sclera, and cornea. Anterior chamber, vitreous,
and fundus examinations are also important.
slit lamp examination reveals a crescent-shaped destructive lesion of the
juxtalimbal corneal stroma associated with an epithelial defect, stromal
yellow-white infiltrates composed of inflammatory cells, and varying degrees
of corneal stromal thinning (minimal to full thickness) adjacent to the limbus.in
severe cases, the peripheral cornea is progressively destroyed
circumferentially and centrally.
PUK accompanied by necrotizing scleritis almost always indicates the presence
of a potentially lethal systemic disease.The anterior chamber should be
evaluated for depth and inflammation.A posterior segment examination is
typically indicated to help determine the underlying etiology.

10. Examination of lids
Blepharitis
Telengiectasis (rosaecae)
Posterior segment examination
Posterior scleritis
Vasculitis of CVD

11. Ocular Investigation
Corneal scraping/culture
Conjunctival biopsy
Removes source of collagen
Diagnosis of CVD fibrinoid
necrosis,granulomas,vasculitis
Diagnosis of Moorens justifies immune suppression in
occult systemic disease

12. Systemic Investigations
CBC
ESR
CRP
URINE ANALYSIS
RF
ANA (SLE/RA)
C ANCA (96% WG)
ANTI-ds DNA(SLE)
C3/C4 LEVELS
CIRCULATING
IMMUNE COMPLEXES
CHEST X-RAY
SINUSES (X-RAY / CT
SCAN)
HEPATITIS B,C Ag

13. Medical therapy
 Ocular
Preservative free artificial tears, closure of puncta with plugs
or cautery, and bandage soft contact lenes are used to treat
associated dry eye and promote epithelialization of the ulcer.
Cyanoacrylate adhesive may be applied to the ulcer bed to
limit ulceration in cases of impending perforation. This may
also prevent influx of white blood cells from the tear film.
Amniotic membrane.
If the perforation is in the very periphery of the cornea, you
can create a conjunctival bridge over it
Topical antibiotics are used to prevent bacterial superinfection

14.  Systemic
Systemic immunosuppression is often required to control ocular
inflammation. Initial treatment initially is with steroids in the form of
prednisone (1 mg/kg/day) or methylprednisolone (1 g/day x 3 days).
Steroid sparing agents are indicated in the case of impending
perforation, disease uncontrolled with steroids, or patients with
associated rheumatoid arthritis as they are at increased risk for
vascular events. Steroid sparing agents include antimetabolites such as
methotrexate, azathioprine, or mycophenolate mofetil, T cell inhibitors
such as cyclosporine or tacrolimus, alkylating agents such as
cyclospophosphamide and chlorambucil, and biologic agents such as 5-
infliximab and rituximab.systemic tetracyclin

15. Medical follow up
Medications and dosages are adjusted based on the level of
clinical response. Systemic medications may be managed in
collaboration with a Rheumatologist or other medical specialist

16. surgical intervention
 Indications for surgical intervention include
 corneal perforation or excessive corneal thinning with
impending perforation. Surgical options include
lamellar, penetrating or crescentric keratoplasty, as well as
possible corneo-scleral keratoplasty with a partial thickness
scleral resection in cases of sceral melting.

 Resection of the conjunctiva adjacent to the area of peripheral
ulcerative keratitis may also be performed to limit
inflammation originating from the conjunctiva.

17. Surgical follow up
Close follow up after surgical intervention is necessary. Patients
should be monitored for recurrence of disease as well as infection
or rejection of the graft

18. Complications
Complications include infection and perforation of the ulcer.
Prognosis
Visual prognosis is related to the severity of disease. Patients with
associated systemic disease have an increased mortality rate
from vascular events

19. Thinning in the 'Quiet' Eye
Dellen
are localized areas of thinning, or drying, of the peripheral cornea. Dellen are
usually located adjacent to an area of tissue swelling, tissue growth,
inflammation, or eyelid abnormality. These abnormalities may alter the eye's
normal ability to spread the tear layer uniformly

20. Furrow degeneration:
usually asymptomatic,may occur as an idiopathic condition in elderly as a lucid
area separating corneal arcus from the limbus.Epithelium is intact, No
vascularization,Corneal thinning may occur .May be associated with systemic
diseases such as rheumatoid arthritis..
No treatment is required in idiopathic degenerations

21. Pellucid marginal degeneration
have severe thinning, usually inferiorly, within a couple of millimeters of the
limbus. Though there's no redness, pain or inflammation, it causes significant
irregular astigmatism, so the patient tends to complain of a slow, progressive
worsening of vision." On topography, PMD will have an area of inferior
steepening that resembles a crab claw, physicians note. "For PMD, glasses
sometimes help, though the management typically involves a rigid gas
permeable contact lens or a hybrid lens

22. Terrien's marginal degeneration.
This presents as a marginal furrow, usually bilateral, and is most common in
men between 20 and 40 years of age. It starts as a non-ulcerated area of
thinning located superiorly, and it slowly progresses from there. "You'll see
vascularization in addition to the thinning, often with a leading edge of lipid,"
However, the epithelium is also intact with this condition. The thinning can be
progressive, and can progress circumferentially or centrally. And, since it starts
superiorly, the patient usually gets against-the-rule astigmatism.

23. Thinning in the 'Hot' Eye
no treatment for the thinning, you can manage the astigmatism with glasses or,
failing that, RGPs or hybrid lenses
you first assess the defect's size, location and whether it's associated with a
hypopyon,""None of the immune conditions cause a hypopyon . if there's a hy-
popyon, it's a bacterial infection until proven otherwise. "Scrape it, culture it .
a non-infectious peripheral ulcerative keratitis, however, first suspect
rheumatoid arthritis or another autoimmune condition such as wegner
granulomatosis,hepatitis
If Investigation is negative so think of mooren ulcer

24. Mooren's ulcer, or a peripheral ulcerative keratitis of unknown
etiology.
"Mooren's is typically more chronic, progressive and very painful,.
"It will begin in the periphery and spread both circumferentially and
centripetally. The key sign is that there will be a leading, undermined edge of
de-epithelialized tissue. There will also usually be blood vessels crossing the
edge.there's also a milder form of Mooren's ulcer that's more limited and
actually responds well to medical therapy consisting of lubrication and low-
dose steroids and tarsorraphy

25. THANK YOU