Skin vasculitis during Creutzfeldt-Jakob's disease.

We report the case of a 72-year-old woman with sporadic Creutzfeldt-Jakob's disease who presented a large purplish erythematous and edematous lesion, with subsequent bullous detachment on the anterior right thigh. The lesion rapidly evolved into an ulcer covered by a blackish necrotic eschar. Histological examination showed intense necrotizing leukocytoclastic vasculitis in the deep and middle dermis. Direct immunofluorescence revealed C3 and IgM deposits around vessels of the middle and deep dermis. The diagnosis of sporadic Creutzfeldt-Jacob's was confirmed post-mortem by immunoblotting on frozen brain tissue which showed pathologic proteinase-resistant prion-related protein isoform glycotype 2A. In the literature, only two cases of Creutzfeldt-Jakob's disease and cutaneous manifestations are reported.