RESUMO

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.

Assuntos

Ataxia Telangiectasia/complicações , Granuloma/etiologia , Dermatopatias/etiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Granuloma/patologia , Humanos , Lactente , Masculino , Dermatopatias/patologia