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VIGNETTES
Granulomatous Dermatitis in Common Variable Immunodeficiency With Functional T-Cell Defect
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An 18-month-old boy was referred to the pediatrics unit of our hospital for recurrent febrile diseases since his sixth month of life. Findings from repeated blood tests showed a marked reduction of circulating IgG and IgA levels (IgG, 336 mg/dL; IgA, 7 mg/dL; and IgM, 192 mg/dL) associated with a slight relative reduction of circulating T-lymphocyte level (57%; reference range, 59.4%-84.6%) in the context of a normal blood cell count. The baby was diagnosed as having common variable immunodeficiency (CVID) associated with a functional defect of T-lymphocytes, as demonstrated by a raised apoptotic response and a reduced proliferative response to mitogens. At age 2 years, we observed a cutaneous eruption of violaceous, nontender papules and plaques over the check, arms, and thighs and buttocks (Figure 1A-C). A biopsy specimen showed heavy granulomatous infiltrate consisting of lymphocytes surrounding epithelioid histiocytes and sparse giant cells without caseating necrosis (Figure . . . [Full Text of this Article]
AUTHOR INFORMATION
Carlo Mazzatenta, MD;
Patrizia Martini, MD;
Laura Luti, MD;
Lucia Matteucci, MD;
Raffaele Domenici, MD
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