S EZARY1 DESCRIBED A SYNDROME manifested by edematous and pigmented erythroderma, leonine facies, lymphadenopathy, and abnormal circulating cells. He considered these abnormal cells to be giant histiocytes which probably originated in the skin, then entered the circulating blood, creating a leukemic state. In this report, the ultrastructural features of the S#{233}zarycell in peripheral blood, ...

Omenn Syndrome (OS) is a rare autosomal recessive form of severe combined immunodeficiency characterized by failure to thrive, erythroderma, persistent diarrhea, lymphadenopathy, hepatosplenomegaly; severe recurrent infections [1]. The syndrome may be accompanied by normal or elevated levels of IgE, lymphocytosis, eosinophilia and hypogammaglobinemia [1]. The syndrome, also known as familial re...