Cutaneous manifestations in pediatric patients with primary immunodeficiencies

Abstract

Primary immunodeficiencies comprise a group of over 300 genetic defects which lead to aberrant development and functioning of different components of the immune system. Skin manifestations in these pathologies are common, affecting 35 - 70 % of patients with primary immunodeficiency diagnosis. To present the casuistic of children with diagnosis of primary immunodeficiencies and cutaneous manifestations studied in the División Inmunología Hospital General de Niños Pedro de Elizalde, Argentina, during the last 10 years. A retrospective cohort study comprising 179 medical records of patients with primary immunodeficiency diagnosis was conducted. Nineteen primary immunodeficiencies with different frequency were observed, and 25 out of 179 children (14 %) presented cutaneous involvement. Seven patients with hyper IgE syndrome, three with selective immunoglobulin A deficiency, three with combined immunodeficiency and three with ataxia telangiectasia presented the highest incidence of dermatological
intercurrences. Infectious etiology of cutaneous symptoms was observed in 36 % of patients, allergic etiology in 48% and autoimmune etiology in 16 %. Increased IgE and decreased IgA immunoglobulins serum levels were observed. Decreased percentual levels of T lymphocytes and increased percentual levels of B lymphocytes were also recorded. The median age of the first dermatological manifestation in 84 % of the children studied was 5 months (range 1- 108), an age significantly lower (p < 0.05) than the median age of primary immunodeficiency diagnosis, 33 months (range 3 -168). Conclusion: Cutaneous involvement with different frequency and clinical characteristics are observed in primary immunodeficiencies. Recognition of skin manifestations together with immunological evaluation.