Cutaneous markers of primary immunodeficiency diseases in children

Primary immunodeficiency diseases (PIDs) are rare but important conditions found predominantly in children, We studied PIDs in a large pediatric hospi tal, their association with cutaneous alterations, and the importance of cu taneous alterations as diagnostic markers. Among 382,383 pediatric patients , 130 (0.0003%) had a PID: humoral in 27, cellular and combined in 18, phag ocytic in 37, and associated with major defects in 45, An average of two cu taneous alterations were present in 90 (69%) patients: infections in 80, ec zema-dermatitis in 38, and miscellaneous in 57, In 71 (79%) patients the cu taneous alterations preceded and were the basis for the clinical immunologi c diagnosis. Only two PIDs were not associated with cutaneous lesions.