Omenn syndrome in an infant with IL7RA gene mutation.

Giliani, Silvia; Bonfim, Carmen; de Saint Basile, Genevieve; Lanzi, Gaetana; Brousse, Nicole; Koliski, Adriana; Malvezzi, Mariester; Fischer, Alain et al. · J Pediatr · 2006

case_report · Level V

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Abstract

Omenn syndrome (OS) is a rare combined immunodeficiency characterized by erythroderma, lymphadenopathy, and autoimmune manifestations. Most cases are due to mutations in the RAG genes. We report a case of OS due to mutations of IL7RA, thus defining Omenn syndrome as a genetically heterogeneous condition.

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