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Article

Omenn syndrome: a lack of tolerance on the background of deficient lymphocyte development and maturation.

Current opinion in rheumatology - 1 Jul 2006

Hönig Manfred, Schwarz Klaus

Abstract excerpt

PURPOSE OF REVIEW: Omenn syndrome is a rare inherited primary immunodeficiency characterized by severe combined immunodeficiency in combination with autoimmune features leading to squamous erythrodermia, alopecia, lymphadenopathy, hepatosplenomegaly, and intractable diarrhea. Recent advances include characterizing the genetic basis of the syndrome and integrating the genetic defects into knowledge of tolerance...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 16763459
DOI: 10.1097/01.bor.0000231907.50290.6f

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Omenn syndrome: a lack of tolerance on the background of deficient lymphocyte development and maturation.PMID 16763459Read ↗

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Reduced central tolerance in Omenn syndrome leads to immature self-reactive oligoclonal T cellsSemantic proximity Read ↗

Omenn syndrome caused by a novel homozygous mutation in recombination activating gene 1.Semantic proximity Read ↗

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