Autoimmune keratolysis in a patient with leucocytoclastic vasculitis: unusual erythema elevatum diutinum with granulomatous pattern

摘要

Purpose: Leucocytoclastic vasculitis is an immune complex-mediated, small vessel disease, that is characterized clinically by the presence of palpable purpuric lesions, most often in association with rheumatic diseases. Ocular manifestations are rare. We describe a patient with an unusual granulomatous pattern of EED associated with autoimmune keratolysis. Case Report: A 64-year-old man with decreased visual acuity and nodular lesions in both hands. Ocular exami-nation presented bilateral superior corneal melting with perfuration in left eye and conjunctival thickness in both eyes, in association with severe inflammatory reaction. His-topathologic examination of the conjunctiva revealed gra-nulomatous vasculitis with neutrophilic infiltrate, giant cells and fibroblastic proliferation. A punch biopsy from skin showed similar characteristics suggesting EED, however there was no giant cell. Conclusion: To our knowledge, autoimmune keratolysis secondary to cutaneous leucocytoclastic vasculitis (EED) has not previously been described, neither did we find a des-cription about granulomatous reaction (from conjunctiva) in EED.