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Omenn Syndrome — SCE Dermatology MCQ

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HardPaediatric DermatologyOmenn SyndromeSCE Dermatology

An 8-month-old has erythroderma, failure to thrive, recurrent infection, lymphadenopathy, hepatosplenomegaly, eosinophilia and raised IgE. Blood contains oligoclonal activated T cells. Which diagnosis best integrates these findings?

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Correct answer: B — Omenn syndrome

Hypomorphic RAG variants can permit expansion of a restricted, autoreactive T-cell repertoire, producing the erythroderma, lymphoid enlargement, eosinophilia and high IgE of Omenn syndrome. The presence of T cells does not exclude a severe combined immunodeficiency phenotype because they are oligoclonal and dysfunctional.

Reference: https://academic.oup.com/bjd/pages/bad-guidelines