Suppressor cell dysfunction and necrotizing lesions in a child.
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.
D000936:Antigen-Antibody Complex / Q000032:analysis; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology* / Q000473:pathology; D007223:Infant; D008214:Lymphocytes / Q000276:immunology; D009336:Necrosis; D012867:Skin / Q000473:pathology*; D013203:Staphylococcal Infections / Q000276:immunology; D050378:T-Lymphocytes, Regulatory / Q000276:immunology*
D000936:Antigen-Antibody Complex