Peripheral Ulcerative Keratitis

Dr Md Ferdous IslamDepartment of Ophthalmology

CMH,Dhaka

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

Types • Marginal keratitis• Mooren ulcer• Terrien marginal degeneration• Associated with systemic autoimmune

diseases• Dellen

Mooren Ulcer

• Rare autoimmune disease

• Characterized by– progressive, – peripheral, – circumferential, – stromal corneal ulceration – with later central spread

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

Symptoms and signs

Peripheral ulcerationinvolving 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

Local peripheral ulceration

Undermining

Circumferential

Healing

Complications• Severe astigmatism due to extensive vascularization &

fibrosis

• Perforation following minor trauma

• Secondary bacterial infection

• Cataract

• Glaucoma

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

Management• Topical – Steroids – Cyclosporin (weeks to show significant effect)– Artificial tears – Collagenase inhibitors (acetylcystine)

• bandage contact lenses may reduce discomfort and promote epithelial healing

• 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

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

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

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

Signs

Crescentic ulcerationwith an epithelial defect, thinning and stromal infiltration at the

limbusSpread is circumferential and occasionally centralin contrast to Mooren ulcer, extension into the sclera may occur

Limbitis, episcleritis or scleritis are usually presentContact lens corneaPerforationRheumatoid paracentral ulcerative keratitis (PCUK)

punched-out more centrally located lesion with little infiltrate in a quiet eye

Perforation can occur rapidly

Crescentic ulceration

Contact lens cornea

PCUK

Management

Principally with systemic immunosuppression in collaboration with a rheumatologist

TopicalArtificial tears (preservative-free)Antibiotics as prophylaxis Steroids may worsen thinning so are generally avoided

SystemicSteroids (via pulsed IV administration) are used to control

acute disease, with immunosuppressive therapy and biological blockers for longer-term management

Tetracycline for its anticollagenase effectSurgical management is generally as for Mooren ulcer, including

conjunctival excision if medical treatment is ineffective

Terrien Marginal Degeneration

• Idiopathic thinning of the peripheral cornea• Young adult to elderly patients• Uncommon• 75% males• Usually bilateral

Symptoms

• Asymptomatic • gradual visual deterioration can occur due to

astigmatism• A few patients experience episodic pain and

inflammation

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 zoneno epithelial defect, and on cursory examination the condition may

resemble arcus senilisSlowly 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 traumaPseudopterygia sometimes develop

Pseudopterygium

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

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

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

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

Treatment

• Weak topical steroid• May be combined with a topical antibiotic• Tetracycline orally• For children , breastfeeding and pregnancy

erythromycin• Treatment of blepheritis

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

Ref

• Kanski’s Clinical Ophthalmology A Systemic Approach by Brad Bowling. 8th Edition

• American Academy of Ophthalmology

Thank You