2. Introduction
• Peripheral Ulcerative Keratits (PUK) is a group
of inflammatory diseases whose final
common pathway is peripheral corneal
thinning
• Thinning and/or ulceration preferentially
affecting the peripheral rather than the
central cornea, and spreading around the
margin
• It should be noted that any cause of corneal
ulceration can affect the periphery
4. Mooren Ulcer
• Rare autoimmune disease
• Characterized by
– progressive,
– peripheral,
– circumferential,
– stromal corneal ulceration
– with later central spread
5. Forms unilateral
• Older patient
• Equal sex distribution
• Slowly progressive
• Responds well to
medical therapy
bilateral
• younger patients
• Indian males
• more aggressive
• likely to need
systemic
immunosuppression
• poorer prognosis
• associated with
severe pain
6. Symptoms and signs
Peripheral ulceration
involving the superficial one-third of the stroma,
variable epithelial loss,
Several distinct foci and subsequently coalesce
Undermined and infiltrated leading edge is characteristic
Limbitis may be present but no scleritis
Progressive circumferential and central stromal thinning
Vascularization involving the bed of the ulcer up to its leading edge but
not beyond
The healing stage is characterized by thinning, vascularization and
scarring
11. Complications
• Severe astigmatism due to extensive vascularization &
fibrosis
• Perforation following minor trauma
• Secondary bacterial infection
• Cataract
• Glaucoma
12. DDx
Mooren ulcer
• not associated with any
systemic abnormality,
except for the
occasional association
with hepatitis C
• No scleral involvement
although associated
conjunctival and
episcleral inflammation
• No clear zone exists
between the ulcer and
the limbus
PUK
• associated with collagen
vascular disease
13. Management
• Topical
– Steroids
– Cyclosporin (weeks to show significant effect)
– Artificial tears
– Collagenase inhibitors (acetylcystine)
• bandage contact lenses may reduce discomfort and
promote epithelial healing
14. • Conjunctival resection
– may be combined with excision of necrotic tissue
– performed if there is no response to topical steroids
– The excised area should extend 4 mm back from the
limbus and 2 mm beyond the circumferential margins
– Keratoepithelioplasty (suturing of a donor corneal
lenticule onto the scleral bed) may be combined to
produce a physical barrier against conjunctival
regrowth and further melting
– Steroids are continued postoperatively
15. Systemic
• Immunosuppression should be instituted earlier for
– bilateral disease
– advanced involvement at presentation
• Systemic collagenase inhibitors such as doxycycline
• Lamellar keratectomy involving dissection of the
residual central island in advanced disease may
remove the stimulus for further inflammation
16. Peripheral Ulcerative Keratitis Associated With
Systemic Autoimmune Disease
• Destructive inflammation of the peripheral cornea
associated with corneal epithelial sloughing and
keratolysis
• The mechanism includes immune complex
deposition in peripheral cornea, episcleral and
conjunctival capillary occlusion with secondary
cytokine release and inflammatory cell recruitment,
the upregulation of collagenases and reduced activity
of their inhibitors
17. Systemic associations
• Rheumatoid arthritis (most common)
– PUK is bilateral in 30% and tends to occur in advanced RA
• Wegener granulomatosis (2nd most common)
– In contrast to RA ocular complications are the initial presentation in
50%
• Other conditions include polyarteritis nodosa, relapsing
polychondritis ,SLE , Churg – Strauss ,Microscopic Polyangiitis,
Inflammatory Bowel Disease
18. Signs
Crescentic ulceration
with an epithelial defect, thinning and stromal infiltration at
the limbus
Spread is circumferential and occasionally central
in contrast to Mooren ulcer, extension into the sclera may
occur
Limbitis, episcleritis or scleritis are usually present
Contact lens cornea
Perforation
Rheumatoid paracentral ulcerative keratitis (PCUK)
punched-out more centrally located lesion with little infiltrate
in a quiet eye
Perforation can occur rapidly
22. Management
Principally with systemic immunosuppression in
collaboration with a rheumatologist
Topical
Artificial tears (preservative-free)
Antibiotics as prophylaxis
Steroids may worsen thinning so are generally avoided
Systemic
Steroids (via pulsed IV administration) are used to control
acute disease, with immunosuppressive therapy and
biological blockers for longer-term management
Tetracycline for its anticollagenase effect
Surgical management is generally as for Mooren ulcer,
including conjunctival excision if medical treatment is
ineffective
23.
24. Terrien Marginal Degeneration
• Idiopathic thinning of the peripheral cornea
• Young adult to elderly patients
• Uncommon
• 75% males
• Usually bilateral
25. Symptoms
• Asymptomatic
• gradual visual deterioration can occur due to
astigmatism
• A few patients experience episodic pain and
inflammation
26. Signs
Fine yellow–white refractile stromal opacities, frequently
associated with mild superficial vascularization,
usually start superiorly,
spread circumferentially
separated from the limbus by a clear zone
no epithelial defect, and on cursory examination the condition
may resemble arcus senilis
Slowly progressive circumferential thinning
results in a peripheral gutter, the outer slope of which shelves
gradually, while the central part rises sharply. A band of lipid is
commonly present at the central edge
Perforation is rare but may be spontaneous or follow blunt
trauma
Pseudopterygia sometimes develop
30. Pseudopterygium vs Pterygium
• Results from corneal burns,
perforation or longstanding
corneal ulcer
• Differentiated by
– Hx of prior inflammation
– Unilateral
– Location
– Configuration other than
the wing shape
– Nonprogressive
– ability to pass a probe
under the neck
31. Management
• Safety spectacles if thinning is significant
• Contact lenses for astigmatism. Scleral or soft lenses
with rigid gas permeable ‘piggybacking’
• Surgery – crescentic or annular excision of the gutter
with lamellar or full-thickness transplantation
32. Marginal Keratitis
• Caused by hypersensitivity reaction against
Staphylococcal exotoxin and cell wall proteins with
deposition of Ag-Ab complex in peripheral cornea
• Leisons are culture negative but S. aureus can be
isolated from lid margin
33. Signs and symptoms
• Chronic Blepheritis
• Subepithelial marginal infiltrates seperated from
limbus by a clear zone
• Conjunctival hyperemia
• Coalescence and circumferential spread
• Little or no AC reaction
• Resolution usually occurs in 1-4 wks, occasionally
there is residual superficial scarring
34. Treatment
• Weak topical steroid
• May be combined with a topical antibiotic
• Tetracycline orally
• For children , breastfeeding and pregnancy
erythromycin
• Treatment of blepheritis
35. Dellen
• Localized corneal disturbance associated with
drying of a focal area
• Usually associated with an adjacent elevated
lesion as pinguecula or large subconjunctival
haemorrhage that impairs physiological
lubrication
36.
37. Ref
• Kanski’s Clinical Ophthalmology A Systemic Approach
by Brad Bowling. 8th Edition
• American Academy of Ophthalmology
