Scleritis in congenital erythropoietic porphyria – infective or inflammatory?

摘要

Congenital erythropoietic porphyria (CEP) is an autosomalrecessive disorder with deficiency of uroporphyrinogen IIIcosynthase. Accumulation of uroporphyrin I is responsiblefor the oxygen.dependent phototoxic dermatological, skeletal,visceral, and ocular damage.[1,2] The dermatological changesinclude skin ulceration and extensive mutilation in the sunexposed area.