Skin Manifestations in Primary Immunodeficient Children
March 2011
in “
Pediatric Dermatology
”
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.
