Case Reports
doi: 10.1016/s0022-3476(05)83043-4.
Cutaneous granulomatous lesions in patients with ataxia-telangiectasia
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- PMID: 1960607
- DOI: 10.1016/s0022-3476(05)83043-4
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Case Reports
Cutaneous granulomatous lesions in patients with ataxia-telangiectasia
J Pediatr.
1991 Dec.
Abstract
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.
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