Omenn Syndrome

Omenn Syndrome is also known as Combined Immune Deficiency with Hypereosinophilia. Both DCLRE1C and RAG disorders can present as Omenn syndrome. Patients have oligoclonal T cells that invade the skin, liver, spleen and gut. Humoral immunity is absent but IgE levels can be high. Exudative erythroderma with lymphadenopathy and hepatosplenomegaly are characteristic.