Cutaneous granulomas in the setting of primary immunodeficiency: a report of four cases and review of the literature

International Journal of Dermatology
Joanna HarpTimothy G Berger

Abstract

Cutaneous granulomas without an identifiable infectious etiology are a rare manifestation of primary immunodeficiency (ID). These cutaneous lesions can be misdiagnosed, often as sarcoidosis, when the skin findings precede the diagnosis of immunodeficiency. We present four cases from our institution and review the literature in order to emphasize the clinical relevance of this association, discuss the histologic and immunohistochemical features, and explore possible pathogenic mechanisms of granuloma formation. We retrospectively reviewed case reports of all patients presenting with cutaneous granulomas in the setting of primary immunodeficiency. Cases with insufficient information to confirm an immunodeficiency state were excluded. Four patients from our clinic were included, for 54 total cases. The majority of cutaneous granulomas are seen in three types of immunodeficiencies: ataxia-telangiectasia, severe combined immunodeficiency, and combined variable immunodeficiency. Twenty-six percent of patients developed cutaneous granulomas prior to their immunodeficiency diagnosis. Histologically, various granulomatous patterns have been described. Immunohistochemistry revealed a CD4+/CD8+ lymphocyte ratio of less than or equal to 1 ...Continue Reading

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Citations

Dec 14, 2016·The Journal of Allergy and Clinical Immunology·Craig D PlattRaif S Geha
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