Arch Dermatol
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Case Reports
Recurrent cutaneous necrotizing eosinophilic vasculitis. A novel eosinophil-mediated syndrome.
Review of skin biopsy specimens showing necrotizing vasculitis revealed three patients with small dermal vessel eosinophilic vasculitis and common clinical features characterized by glucocorticoid responsive pruritic erythematous, purpuric papules and angioedema associated with peripheral blood eosinophilia. Indirect immunofluorescent localization of eosinophil granule proteins, neutrophil granule proteins, and mast cell tryptase, electron-microscopic evaluation and immunoperoxidase staining for vascular cell adhesion molecule type 1, intercellular adhesion molecule type I, endothelial-leukocyte adhesion molecule type 1, and very-late activation antigen type 4 were performed. Eosinophil-active cytokines in serum were evaluated by an eosinophil survival assay. ⋯ We studied three patients whose cutaneous lesions showed small-vessel eosinophilic vasculitis and who presented with recurrent glucocorticoid-responsive pruritic purpuric papules and angioedema. The presence of eosinophil-active cytokines in serum and the expression of vascular cell adhesion molecule type 1 on the endothelium of affected vessels may contribute to the selective adherence and localization of activate eosinophils. Subsequent release of cytotoxic proteins such as major basic protein may result in destruction of the endothelium in this unique syndrome.