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Hailey-Hailey Disease — SCE Dermatology MCQ

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HardBlistering DiseasesHailey-Hailey DiseaseSCE Dermatology

A 40-year-old woman presents with Hailey-Hailey disease affecting her groin. She has tried topical corticosteroids and antibiotics without adequate control. She experiences significant friction-related flares. What novel treatment has shown benefit for refractory Hailey-Hailey?

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Correct answer: C — Botulinum toxin injections to reduce sweating in affected areas

The best answer is “Botulinum toxin injections to reduce sweating in affected areas”. Hailey–Hailey disease is an autosomal-dominant ATP2C1 disorder with variable expression; heat, sweat, friction and infection commonly aggravate flexural disease. The alternatives “Oral Isotretinoin, when the full phenotype supports it”, “Oral Dapsone, within an appropriate UK pathway”, “Topical Sirolimus, after clinicopathological correlation, after specialist assessment”, “Oral Terbinafine, after specialist assessment, when the full phenotype supports it” are clinically adjacent possibilities, but they do not fit the defining morphology, distribution, histopathology, risk signal or management sequence in this stem.

Reference: BAD Hailey-Hailey disease information: https://www.bad.org.uk/pils/hailey-hailey-disease