Skin Manifestations in Primary Immunodeficient Children

    March 2011 in “ Pediatric Dermatology ”
    Waleed Al‐Herz, Arti Nanda
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    TLDR Nearly half of children with primary immunodeficiency disorders showed skin problems, often as the first sign of their condition.
    In a study of 128 children with primary immunodeficiency disorders (PID), 61 patients (48%) exhibited skin manifestations, and in 50 patients (39%), these were the initial symptoms leading to the diagnosis of PID. The most common skin issues were infections (30%) and eczemas (19%), with specific manifestations linked to certain types of PID, such as eczema in hyper IgE syndrome and Wiskott-Aldrich syndrome, erythroderma with alopecia in severe combined immunodeficiency, telangiectasia in ataxia telangiectasia, and partial albinism with silvery hair in Chediak-Higashi syndrome. Autoimmune skin manifestations were also observed in 6% of the cases. The study emphasized the importance of recognizing skin symptoms for the early diagnosis and treatment of PID in children.
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