title: 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.
authors.affiliation: Department of Immunology and Infectious Diseases, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.
authors.forename: Sylvan
authors.initials: S
authors.lastname: Rego
authors.forename: Andrew
authors.initials: A
authors.lastname: Kemp
authors.forename: Melanie
authors.initials: M
authors.lastname: Wong
authors.forename: Paul
authors.initials: P
authors.lastname: Knight
mesh_terms: D000505:Alopecia / Q000209:etiology*; D016026:Bone Marrow Transplantation; D065095:Calcineurin Inhibitors; D016572:Cyclosporine / Q000627:therapeutic use*; D003873:Dermatitis, Exfoliative / Q000188:drug therapy; D005260:Female; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D007231:Infant, Newborn; D008218:Lymphocytosis / Q000188:drug therapy / Q000209:etiology; D009035:Mothers; D016511:Severe Combined Immunodeficiency / Q000188:drug therapy* / Q000628:therapy; D013577:Syndrome; D016559:Tacrolimus / Q000627:therapeutic use; D017211:Treatment Failure
chemical_list: D065095:Calcineurin Inhibitors; D016572:Cyclosporine; D016559:Tacrolimus