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

R. A. Pupo, S. K. Tyring, Sharon Raimer, D. P. Wirt, E. G. Brooks, R. M. Goldblum

Research output: Contribution to journalArticle

25 Citations (Scopus)

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 languageEnglish (US)
Pages (from-to)442-446
Number of pages5
JournalJournal of the American Academy of Dermatology
Volume25
Issue number2 II SUPPL.
StatePublished - 1991

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Exfoliative Dermatitis
Severe Combined Immunodeficiency
Lymphocytosis
Failure to Thrive
Alopecia
Eosinophilia
Anemia
Lymph Nodes
T-Lymphocytes
Phenotype
Skin
Infection
Lymphadenopathy

ASJC Scopus subject areas

  • Dermatology

Cite this

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

In: Journal of the American Academy of Dermatology, Vol. 25, No. 2 II SUPPL., 1991, p. 442-446.

Research output: Contribution to journalArticle

Pupo, R. A. ; Tyring, S. K. ; Raimer, Sharon ; Wirt, D. P. ; Brooks, E. G. ; Goldblum, R. M. / Omenn's syndrome and related combined immunodeficiency syndromes : Diagnostic considerations in infants with persistent erythroderma and failure to thrive. In: Journal of the American Academy of Dermatology. 1991 ; Vol. 25, No. 2 II SUPPL. pp. 442-446.
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