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

doi:10.1016/S0022-3476(05)83043-4

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

Dermatology

Research output: Contribution to journal › Article › peer-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 language	English (US)
Pages (from-to)	917-922
Number of pages	6
Journal	The Journal of pediatrics
Volume	119
Issue number	6
DOIs	https://doi.org/10.1016/S0022-3476(05)83043-4
State	Published - Dec 1991

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health

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title = "Cutaneous granulomatous lesions in patients with ataxia-telangiectasia",

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.",

author = "Paller, {Amy S.} and Massey, {Ruby B.} and Curtis, {M. Anne} and Pelachyk, {John M.} and Dombrowski, {Helene C.} and Leickly, {Frederick E.} and Michael Swift",

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AU - Paller, Amy S.

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AU - Curtis, M. Anne

AU - Pelachyk, John M.

AU - Dombrowski, Helene C.

AU - Leickly, Frederick E.

AU - Swift, Michael

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AB - 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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