Peripheral ulcerative keratitis (PUK) is an inflammatory thinning and ulceration of the cornea as a result of an autoimmune condition. Causative autoimmune diseases include:

• Rheumatoid arthritis
• Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitidies:
  • Granulomatosis with polyangiitis
  • Polyarteritis nodosa
  • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
  • Microscopic polyangiitis
  • Inflammatory bowel disease
• Systemic lupus erythematosus
• Relapsing polychondritis

Of these, rheumatoid arthritis is the most common, resulting in 32%-42% of PUK patients.

PUK affects 3 persons per million per year. There is no predilection by sex, but PUK more commonly affects patients after their fourth or fifth decade.

Patients present with ocular redness, pain, tearing, photophobia, and decreased vision. The classic presentation is a crescent-shaped white ulcer on the peripheral edge of the cornea.

Cases are mostly unilateral but can be bilateral. About 36%-66% of PUK cases can be associated with a scleritis, and a wider incidence of cases can be associated with anterior uveitis. The worst complications of PUK are corneal perforation or scleral perforation from an associated necrotizing scleritis. These complications need immediate surgical intervention to prevent infection and vision loss.