Omenn's syndrome and related combined immunodeficiency syndromes: Diagnostic considerations in infants with persistent erythroderma and failure to thrive

Rafael A. Pupo; Stephen K. Tyring; Sharon S. Raimer; Daniel P. Wirt; Edward G. Brooks; Randall M. Goldblum

doi:10.1016/0190-9622(91)70225-Q

Omenn's syndrome and related combined immunodeficiency syndromes: Diagnostic considerations in infants with persistent erythroderma and failure to thrive

Rafael A. Pupo, Stephen K. Tyring, Sharon S. Raimer, Daniel P. Wirt, Edward G. Brooks, Randall M. Goldblum

Research output: Contribution to journal › Article

25 Scopus citations

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.

Original language	English (US)
Pages (from-to)	442-446
Number of pages	5
Journal	Journal of the American Academy of Dermatology
Volume	25
Issue number	2
DOIs	https://doi.org/10.1016/0190-9622(91)70225-Q
State	Published - Jan 1 1991

ASJC Scopus subject areas

Dermatology

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10.1016/0190-9622(91)70225-Q

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Pupo, R. A., Tyring, S. K., Raimer, S. S., Wirt, D. P., Brooks, E. G., & Goldblum, R. M. (1991). Omenn's syndrome and related combined immunodeficiency syndromes: Diagnostic considerations in infants with persistent erythroderma and failure to thrive. Journal of the American Academy of Dermatology, 25(2), 442-446. https://doi.org/10.1016/0190-9622(91)70225-Q

Omenn's syndrome and related combined immunodeficiency syndromes : Diagnostic considerations in infants with persistent erythroderma and failure to thrive. / Pupo, Rafael A.; Tyring, Stephen K.; Raimer, Sharon S.; Wirt, Daniel P.; Brooks, Edward G.; Goldblum, Randall M.

In: Journal of the American Academy of Dermatology, Vol. 25, No. 2, 01.01.1991, p. 442-446.

Research output: Contribution to journal › Article

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