Cutaneous granulomatous lesions in patients with ataxia-telangiectasia

Amy S. Paller*, Ruby B. Massey, M. Anne Curtis, John M. Pelachyk, Helene C. Dombrowski, Frederick E. Leickly, Michael Swift

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

52 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)917-922
Number of pages6
JournalThe Journal of pediatrics
Volume119
Issue number6
DOIs
StatePublished - Dec 1991

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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