title: Omenn's syndrome and related combined immunodeficiency syndromes: diagnostic considerations in infants with persistent erythroderma and failure to thrive.
abstract: A 4-month-old male infant had a 2-month history of an exfoliative erythroderma and alopecia. Recurrent mucosal infections, diffuse lymphadenopathy, hepatosplenomegaly, lymphocytosis and eosinophilia, anemia, and failure to thrive later developed. Investigation revealed a combined immunodeficiency with T cells of an unusual phenotype in his peripheral blood, skin, and lymph nodes. Our patient's clinical manifestations most closely resemble Omenn's syndrome, a rare form of autosomal recessive combined immunodeficiency.
authors.affiliation: Department of Dermatology, University of Texas Medical Branch, Galveston 77550.
authors.forename: R A
authors.initials: RA
authors.lastname: Pupo
authors.forename: S K
authors.initials: SK
authors.lastname: Tyring
authors.forename: S S
authors.initials: SS
authors.lastname: Raimer
authors.forename: D P
authors.initials: DP
authors.lastname: Wirt
authors.forename: E G
authors.initials: EG
authors.lastname: Brooks
authors.forename: R M
authors.initials: RM
authors.lastname: Goldblum
mesh_terms: D000505:Alopecia / Q000473:pathology; D003873:Dermatitis, Exfoliative / Q000473:pathology; D003937:Diagnosis, Differential; D005183:Failure to Thrive; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000473:pathology; D007223:Infant; D008297:Male; D013577:Syndrome; D013601:T-Lymphocytes / Q000473:pathology