A case of Omenn-like immunodeficiency syndrome.
We report the case of a child with a variant of the Omenn immunodeficiency syndrome. He presented with erythroderma, lymphadenopathy, hepatosplenomegaly, failure to thrive, and recurrent purulent infections. The immunological studies showed marked disturbances in the subpopulations and functions of T lymphocytes, which suggests a defect in T cell differentiation as the cause of the disease.
Morren
M
M
Department of Dermatology, University Hospital, Katholieke Universiteit Leuven, Belgium.
Van Lierde
S
S
Lacquet
F
F
Ceuppens
J L
JL
Delabie
J
J
Dewolf-Peeters
C
C
Degreef
H
H
D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D007223:Infant; D008213:Lymphocyte Activation / Q000276:immunology; D008297:Male; D012871:Skin Diseases / Q000276:immunology*; D016176:T-Lymphocyte Subsets / Q000276:immunology