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February 1981

Suppressor Cell Dysfunction and Necrotizing Lesions in a Child

Author Affiliations

From the Departments of Pediatrics and Biochemistry, Thomas Jefferson University, Jefferson Medical College, Philadelphia.

Arch Dermatol. 1981;117(2):93-98. doi:10.1001/archderm.1981.01650020035021

• A girl had opportunistic infections and was found to have T-cell dysfunction. During a period of months, recurrent staphylococcal infections, polyclonal hyperglobulinemia, eosinophilia, and peripheral, necrotizing, cutaneous lesions developed. Circulating immune complexes were demonstrated, and abnormal suppressor-cell function was found. At age 36 months, the child died of a staphylococcal pneumonia. At postmortem examination, the thymus gland was found to be histologically abnormal, lacking corticomedullary differentiation. We propose that this patient had a syndrome in which lymphocyte abnormalities and dysfunction of suppressor T cells permitted hyperresponsiveness of antibody-forming cells. Large amounts of circulating antibody and immune complexes were formed, and their deposition led to peripheral tissue injury.

(Arch Dermatol 117:93-98, 1981)