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June 2006

Granulomatous Dermatitis in Common Variable Immunodeficiency With Functional T-Cell Defect

Arch Dermatol. 2006;142(6):781-798. doi:10.1001/archderm.142.6.783

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 1D). Staining results for acid-fast bacilli and gram-positive and gram-negative bacteria or fungi were negative. Blood chemistry test results showed a raised level of angiotensin-converting enzymes (210 U/L; reference range, 66-144 U/L), but findings from chest radiography and abdominal ultrasonography were normal. At age 7 years, the child successfully underwent homologous bone marrow transplantation to correct the immune deficit. A few weeks after transplantation, we observed a progressive fainting of cutaneous lesions, which completely disappeared in a few months, leaving slightly atrophic scars. When last seen in January 2005, the patient, aged 11 years, was free of symptoms (Figure 2).

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