Evidence for pathogenic involvementof eosinophils and neutrophilsin Churg-Strauss syndrome

Abstract

Background: Churg-Strauss syndrome (CSS) is a multi-organ disease with tissue and blood eosinophilia. Objective: Our aim was to study eosinophil and neutrophil involvement in CSS. Methods: Eight lesional skin biopsy specimens from 6 patients with CSS and serum and blister fluid from one patient were tested for eosinophil and neutrophil activity. Indirect immunofluorescence on skin specimens used antibodies to eosinophil granule major basic protein (MBP), eosinophil-derived neurotoxin (EDN), and neutrophil elastase (NE). Serum and blister fluid specimens were analyzed for granule protein levels and for eosinophil-activating cytokines. Results: Indirect immunofluorescence showed prominent cellular and extracellular staining for EDN in skin biopsy specimens; MBP staining was less extensive. Five biopsy specimens showed marked cellular NE staining; 4 showed prominent extracellular NE. Serum and blister fluid specimens contained elevated MBP, EDN, and interleukin 5 levels and enhanced eosinophil survival in culture. Granulocyte-macrophage colony-stimulating factor and interleukin 5 were detected in blister fluid. Blister fluid contained more NE than normal serum. Conclusions: Both eosinophils and neutrophils likely participate in skin lesion development in CSS. (J Am Acad Dermatol 2002;47:209-16.)