Peripheral Ulcerative Keratopathies

Peripheral ulcerative keratopathy associated with systemic disease

• Common systemic causes

  • Rheumatoid arthritis
  • Wegener’s
  • Relapsing polychondritis
  • SLE
  • Idiopathic
  • Sarcoidosis
  • Churg-Strauss
  • Type 1 diabetes

• Infectious causes

• Non-infectious

  • Terrien’s
  • Marginal keratitis/blepharitis
  • Pellucid
  • Rosacea
  • Neurotrophic

• Presents with pain, redness and reduced vision

• May be bilateral

• There is peripheral corneal ulceration with stromal thinning and associated limbal inflammation

• There may be sectoral or diffuse scleritis

Management

• Topical lubricants (and consider punctual plugs/cautery)
• Microbiology including corneal scrapings to exclude infection
• Systemic immunosuppression
• Doxycycline
• Oral vitamin C
• Topical steroids (may accelerate keratolysis)
• Cycloplegia
• Globe protection eg. tarsorrhaphy or botox ptosis
• Therapeutic CL
• Gluing
• Surgery: conjunctival recession, amniotic graft, lamellar keratoplasty

Mooren’s ulcer

• A rare form of PUK
• Autoimmune
• Associated with hepatitis C

Two forms:

• Limited: affects middle-aged to elderly Caucasian patients, unilateral and responsive to treatment
• Aggressive: affects young African patients (?antigen-antibody reaction to helminthic toxins), bilateral, resistant to treatment and higher risk of perforation

Clinical features

• Pain, photophobia, reduced visual acuity
• Progressive peripheral ulceration
• Leading edge to ulcer undermining the epithelium
• Grey infiltrate at the leading edge
• Progresses centrally and circumferentially
• Stromal melt
• No limbal clear zone (cp. Marginal keratitis and Terrien’s)
• No scleritis (cp. Typical PUK)
• Immunoglobulin, complement and plasma cells in the conjunctiva

Tests

• Exclude hepatitis C
• Systemic review of BP, FBC, ESR, CRP, U&Es, LFTs, glucose, HbA1c, vasculitis screen, ANA, ANCA, dsDNA, ACE, urinalysis, CXR

Management

• Topical steroids
• Systemic immunosuppression: steroids, cyclophosphamide, ciclosporin A
• Topical antibiotics
• Cycloplegia
• BCL +/- glue
• Surgery: lamellar keratoplasty
• Prognosis: typically burns out over 6-18 months but can cause blindness

Terrien marginal degeneration

• Asymmetrically bilateral
• Superior thinning then spreads circumferentially
• Pannus traverses the area of thinning
• A lipid line is seen at the end of the pannus, posterior to the area of thinning
• Clear interval between limbus and ulcer (cp. With Mooren’s)
• Onset 2-3rd decade
• M>F
• No loss of epithelium (therefore not painful)
• Against the rule astigmatism: steepening occurs 90 degrees away
• A paediatric form exists: Fuch’s superficial marginal keratitis

Management:

• Glasses/CLs for astigmatism
• Crescentic lamellar transplantation if perforation occurs (rare)