Copyright 2006 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2006
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).
Mazzatenta C, Martini P, Luti L, Matteucci L, Domenici R. 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