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The Capital Region of Denmark - a part of Copenhagen University Hospital
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Sjælden immundefekt bag svær atopisk dermatitis i barnealderen

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Two children are presented with autosomal recessive hyper IgE syndrome caused by a mutation in the dedicator of cytokinesis 8 gene (DOCK8). The manifestations are typically severe atopic dermatitis, food allergies, elevated serum IgE concentration, viral skin infections and risk of malignancies. DOCK8 deficiency was first reported in 2009, following the death of the oldest sibling. The youngest sibling was cured after allogenic stem cell transplantation. This case report illustrates the need of awareness of primary immunodeficiency in children with atypical manifestation of atopic dermatitis in combination with recurrent infections.

Translated title of the contributionSevere atopic dermatitis caused by rare immunodeficiency in childhood
Original languageDanish
JournalUgeskrift for Laeger
Volume177
Issue number51
Pages (from-to)V06150498
ISSN0041-5782
Publication statusPublished - 14 Dec 2015

ID: 46235410