Recurrent cutaneous necrotising eosinophilic vasculitis

Recurrent cutaneous necrotising eosinophilic vasculitis (RCNEV) is a rare disease that was first described in 1994. We report a case of RCNEV treated with corticosteroid, and 18 cases that we identified in the literature. Our review of the literature shows that RCNEV was frequently identified in middle-aged females from Asia and usually presents as erythematous to purpuric papuloplaques, angio-oedema on the extremities, as well as peripheral eosinophilia. Histopathologically, RCNEV is characterised by exclusively eosinophilic infiltration around the vascular plexus, the absence of leukocytoclasis and fibrinoid degeneration of vascular walls. Although, RCNEV responds to corticosteroid treatment, relapses have occurred during dose tapering. We also discuss the mechanisms of vascular destruction, the differential diagnosis and steroid-sparing therapies for RCNEV.