Omenn syndrome: therapeutic effects of cyclosporin.
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.
Rego
Sylvan
S
Department of Immunology and Infectious Diseases, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.
Kemp
Andrew
A
Wong
Melanie
M
Knight
Paul
P
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
D065095:Calcineurin Inhibitors; D016572:Cyclosporine; D016559:Tacrolimus