Omenn syndrome: Therapeutic effects of cyclosporin

Abstract

Omenn syndrome is a severe combined immunodeficiency with features of generalised erythroderma alopecia and evidence of Th2 inflammation (eosinophilia and raised IgE). We describe a differential effect of 2 calcineurin inhibitors, cyclosporine A (CsA) and tacrolimus, with CsA rapidly improving the erythroderma and lymphocytosis but tacrolimus having little effect. © 2006 Paediatrics and Child Health Division (Royal Australasian College of Physicians).