Cutaneous granulomatous lesions in patients with ataxia-telangiectasia.
Ataxia-telangiectasia is a genetic syndrome with progressive cerebellar ataxia, oculocutaneous telangiectasias and other skin manifestations, variable immune system defects, chronic progressive sinopulmonary disease, and a high incidence of cancer. Cutaneous granulomas developed and persisted in eight patients with ataxia-telangiectasia, despite treatment with intravenously administered immune globulin, topical antibiotic therapy, and potent topical corticosteroid therapy. We were unable to identify an infectious agent; the granulomas may develop in an attempt to localize antigen in the presence of a dysfunctional immune system.
Paller
A S
AS
Department of Pediatrics, Northwestern University Medical School, Chicago, Illinois.
Massey
R B
RB
Curtis
M A
MA
Pelachyk
J M
JM
Dombrowski
H C
HC
Leickly
F E
FE
Swift
M
M
D000293:Adolescent; D000328:Adult; D001260:Ataxia Telangiectasia / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006099:Granuloma / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007223:Infant; D008297:Male; D012871:Skin Diseases / Q000209:etiology* / Q000473:pathology