Abstract
Both autosomal recessive and autosomal dominant forms of hyper IgE syndrome (HIES) present with elevated IgE and recurrent skin infections Unlike the autosomal dominant HIES, patients with autosomal recessive HIES have a greater risk of atopy, viral skin infections, and malignancy While HIES clinical criteria help make a quick diagnosis and start therapy, a definitive diagnosis is pending on through genetic testing HSCT is now considered standard of care for DOCK8 deficiency given an appropriate donor is available
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