Zhi-Yong Zhang et al.
Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology, 22(5), 482-487 (2011-07-21)
Omenn syndrome (OS) is a rare autosomal recessive genetic disorder and presents symptoms of severe combined immunodeficiency characterized by erythrodermia, eosinophilia, hepatosplenomegaly, lymphadenopathy, and elevated serum IgE levels. OS has been found to be caused by mutations in RAG1 or