TY - JOUR
T1 - Omenn's syndrome and related combined immunodeficiency syndromes
T2 - Diagnostic considerations in infants with persistent erythroderma and failure to thrive
AU - Pupo, Rafael A.
AU - Tyring, Stephen K.
AU - Raimer, Sharon S.
AU - Wirt, Daniel P.
AU - Brooks, Edward G.
AU - Goldblum, Randall M.
PY - 1991/1/1
Y1 - 1991/1/1
N2 - 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.
AB - 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.
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U2 - 10.1016/0190-9622(91)70225-Q
DO - 10.1016/0190-9622(91)70225-Q
M3 - Article
C2 - 1832695
AN - SCOPUS:0025856973
SN - 0190-9622
VL - 25
SP - 442
EP - 446
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
IS - 2
ER -