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 journalArticle

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 languageEnglish (US)
Pages (from-to)442-446
Number of pages5
JournalJournal of the American Academy of Dermatology
Volume25
Issue number2
DOIs
StatePublished - Jan 1 1991

ASJC Scopus subject areas

  • Dermatology

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