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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