Cutaneous granulomas in children with combined immunodeficiency

Perforating Neutrophilic and Granulomatous Dermatitis of the Newborn—A Clue to Immunodeficiency

“…A distinct, noninfectious, granulomatous condition may appear in different types of immunodeficiency, mainly common variable immunodeficiency, ataxia‐telangiectasia, and hypogammaglobulinemia. A variable spectrum of granulomatous changes has been reported histologically, including nonnecrotizing epithelioid granulomas (sarcoid‐like), caseating (tuberculoid) granulomas or necrobiotic granulomas (10–14), but diffuse, interstitial, suppurative granulomas have not been reported. Granulomas of immunodeficiency affect children and adults, but have not been described in newborns.…”

Cutaneous Granulomas with Predominantly CD8+ Lymphocytic Infiltrate in a Child with Severe Combined Immunodeficiency

“…4 They appear mainly in an acral distribution and are accompanied by inversed circulating CD4 to CD8 ratio. 3,4 Pathogenesis is unknown, but it has been proposed that in a state of humoral deficiency and recurrent inflammation, cellular immune components overcompensate and form granulomas. 5 The predominance of CD8 + T cells in our patient implies a cell-mediated immune response without the intervention of the T-helper cells, probably attributable to the immunologic impairment.…”

Acneiform Granulomatous Folliculitis in a Patient with Interferon-γ Deficiency: Clearing with Empiric Antimycobacterial Therapy

“…In immunodeficient patients, treatment with anti-inflammatory agents potentiates their increased risk of infections. 18 Without data to confirm a specific etiology, the risk-to-benefit ratio for empiric treatment favors an antimicrobial approach. The choice of an appropriate antimicrobial agent can be narrowed, on the basis of the nature of the immunodeficiency.…”