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A Novel Eosinophil-Mediated Syndrome

Ko-Ron Chen, MD; Mark R. Pittelkow, MD; W. P. Daniel Su, MD; Gerald J. Gleich, MD; Walter Newman, PhD; Kristin M. Leiferman, MD

Arch Dermatol. 1994;130(9):1159-1166.

Abstract
Background and Design
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 1, 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.

Observations
Eight skin biopsy specimens from the three patients all showed small-vessel necrotizing vasculitis with exclusive eosinophil infiltration. Ultrastructural study demonstrated degenerating eosinophils and eosinophil granules in proximity to damaged endothelium. The affected small vessels showed marked depo- sition of the toxic eosinophil granule major basic protein in the vessel walls and expression of vascular cell adhesion molecule type 1 and intercellular adhesion molecule type 1 on the endothelium with adherence of very-late activation antigen type 4—positive eosinophils; E-selectin staining was negative. The presence of interleukin 5 in serum available from one patient was detected by an eosinophil survival assay.

Conclusions
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 activated eosinophils. Subsequent release of cytotoxic proteins such as major basic protein may result in destruction of the endothelium in this unique syndrome.

(Arch Dermatol. 1994;130:1159-1166)

Author Affiliations
From the Department of Dermatology (Drs Chen, Pittelkow, Su, and Leiferman) and Division of Allergic Diseases and Internal Medicine and Department of Immunology (Dr Gleich), Mayo Clinic and Mayo Foundation, Rochester, Minn, and Otsuka Inc, Rockville, Md (Dr Newman). Drs Chen and Newman are now with the Kawasaki City (Japan) Hospital and Leukosite, Boston, Mass, respectively.

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