Synonym: vasculitic or rheumatoid peripheral ulcerative keratitis, PUK
Features:
(1) rheumatoid arthritis with microangiopathic vasculitis
(2) often ANCA-positive
(3) necrotizing ulceration of the peripheral cornea
(4) severe ocular pain and photophobia
(5) rapidly progressive keratolysis with perforation
(6) complete visual loss if untreated
It may be triggered by surgery or trauma to the eye. A patient with rheumatoid arthritis should be monitored for this complication following eye surgery. A patient with a history of PUK should be evaluated for immunosuppression prior to eye surgery.
Associated findings:
(1) scleromalacia perforans
(2) systemic manifestations of vasculitis
(3) superinfection may occur
Differential diagnosis:
(1) PUK associated with other types of vasculitis (SLE, polychondritis, etc)
Therapy involves administration of cyclophosphamide or infliximab.
