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Urticaria Pigmentosa — SCE Dermatology MCQ

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HardPaediatric DermatologyUrticaria PigmentosaSCE Dermatology

A 6-year-old with widespread urticaria pigmentosa has recurrent flushing and presyncope. Examination reveals hepatosplenomegaly and baseline serum tryptase is persistently raised. Which interpretation is most appropriate?

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Correct answer: D — Refer for systemic mast-cell disease evaluation and formulate an emergency mediator-release plan

The best answer is “Refer for systemic mast-cell disease evaluation and formulate an emergency mediator-release plan”. Flush, presyncope, hepatosplenomegaly and raised baseline tryptase are red flags for extracutaneous mast-cell disease, so referral and individual emergency planning are appropriate. Deferring review until puberty is unsafe; paired acute and baseline tryptase testing at a future episode does not address the current abnormalities. Cytoreduction and CD1a testing do not follow from these findings.

Reference: BAD urticaria pigmentosa information: https://www.bad.org.uk/pils/urticaria-pigmentosa