Autoimmune keratolysis in a patient with leukocytoclastic vasculitis - Unusual erythema elevatum diutinum with granulomatous pattern

Purpose. Leukocytoclastic vasculitis (LCCV) is an immune complex-mediated, small vessel disease that is clinically characterized by the presence of pa lpable purpuric lesions, most often in association with rheumatic diseases. Ocular manifestations of LCCV are rare. Methods. We describe a patient wit h an unusual granulomatous pattern of erythema elevatum diutinum (EED) asso ciated with autoimmune keratolysis. Results. We studied a 64-year-old man w ith decreased visual acuity and nodular lesions in both hands. Ocular exami nation revealed bilateral superior corneal melting with perforation in the left eye and conjunctival thickening in both eyes, in association with a se vere inflammatory reaction. Histopathologic examination of the conjunctiva revealed granulomatous vasculitis with neutrophilic infiltrate, giant cells , and fibroblastic proliferation. A punch biopsy taken from his skin showed similar characteristics that suggested EED; however, there were no giant c ells. Conclusion, To our knowledge, autoimmune keratolysis secondary to cut aneous LCCV (EED) has not been described previously, and there has been no description of granulomatous reaction (in the conjunctiva) in EED.