CVID-associated Granulomatous Dermatosis Resembling Sarcoidosis

Main Article Content

Jeanette R Zambito http://orcid.org/0000-0002-2178-8998
Pooja R Shah
Glynis A Scott
Andrew Evans
Lisa A Beck

Keywords

cvid, granulomatous dermatosis, sarcoidosis, immunodeficiency

Abstract

Common variable immunodeficiency (CVID) is a disorder of the adaptive immune system predominantly characterized by hypogammaglobulinemia and variable T-cell abnormalities. Patients classically present with recurrent sinopulmonary infections, but interstitial lung disease, autoimmunity, and lymphoproliferative disorders are also common. Approximately 10% of CVID patients are reported to have noncaseating granulomatous disease that is indistinguishable from sarcoidosis on pathology; it most commonly affects the lungs, lymph nodes, and liver.

We present the case of a 75-year-old male with known CVID who presented with granulomatous dermatosis on the trunk and extremities in the setting of progressive cognitive impairment, new-onset cough with ground glass opacities on chest computed tomography in the setting of stable mediastinal lymphadenopathy, and the presence of granulomas on prior bone marrow biopsy, initially suggesting the diagnosis of sarcoidosis. Though clinically and histologically similar, it is important to make the distinction between CVID-associated granulomatous disease and sarcoidosis in order to select appropriate treatment.

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