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 hospital, their association with cutaneous alterations, and the importance of cutaneous alterations as diagnostic markers. Among 382,383 pediatric patients, 130 (0.0003%) had a PID: humoral in 27, cellular and combined in 18, phagocytic in 37, and associated with major defects in 45. An average of two cutaneous alterations were present in 90 (69%) patients: infections in 80, eczema-dermatitis in 38, and miscellaneous in 57. In 71 (79%) patients the cutaneous alterations preceded and were the basis for the clinical immunologic diagnosis. Only two PIDs were not associated with cutaneous lesions.
Berron-Ruiz
A
A
Department of Dermatology and Immunology, National Institute of Pediatrics, Mexico City, Mexico.
Berron-Perez
R
R
Ruiz-Maldonado
R
R
D000293:Adolescent; D017677:Age Distribution; D015415:Biomarkers / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis* / Q000453:epidemiology*; D015994:Incidence; D007223:Infant; D008297:Male; D008800:Mexico / Q000453:epidemiology; D012189:Retrospective Studies; D012307:Risk Factors; D012680:Sensitivity and Specificity; D012871:Skin Diseases / Q000175:diagnosis* / Q000276:immunology*
D015415:Biomarkers