Development of cutaneous granulomas in 8 patients with ataxia-telangiectasia is reported from the Departments of Pediatrics and Dermatology, Northwestern University Medical School, Chicago, IL; the Hospital for Sick Children, Toronto, Canada; Henry Ford Hospital, Detroit, MI; and University of North Carolina, Chapel Hill. The granulomas were atrophic lesions that frequently became encrusted and ulcerative. Their appearance differed significantly from the well circumscribed annular plaques or skin colored subcutaneous nodules of typical granuloma annulare. The lesions were persistent rather than self-limited and no infectious organisms were demonstrated. Treatment with intravenous immune globulin, topical antibiotic therapy and topical corticosteroid therapy were unsuccessful. The authors postulate that the granulomas were an attempt to localize antigen in patients with a dysfunctional immune system. [1]

COMMENT. Patients with ataxia-telangiectasia have dysfunction of humoral and cell-mediated immunity as demonstrated by an immature thymus; absent or small tonsils; low levels of immunoglobulins A, E, G2 and G4; and a poor response to antigenic stimulation.