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Hailey-Hailey Disease — SCE Dermatology MCQ

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HardBlistering DiseasesHailey-Hailey DiseaseSCE Dermatology

A 47-year-old man presents with a cluster of flesh-coloured to yellowish papules arranged in a linear pattern in the left inguinal crease. They have been present for years and occasionally become macerated and malodorous. Biopsy shows papillomatous acanthosis with basophilic cuticle-lined cystic spaces in the dermis consistent with apocrine glands. What is the diagnosis?

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Correct answer: A — Syringocystadenoma papilliferum

The best answer is “Syringocystadenoma papilliferum”. The level of blister cleavage and the distribution of immunoreactants distinguish intraepidermal pemphigus-pattern disorders from subepidermal autoimmune blistering diseases. The alternatives “Hailey-Hailey disease”, “Syringoma”, “Fox-Fordyce disease”, “Apocrine hidrocystoma” are clinically adjacent possibilities, but they do not fit the defining morphology, distribution, histopathology, risk signal or management sequence in this stem.

Reference: BAD pemphigus and blistering disease information: https://www.bad.org.uk/pils/pemphigus-vulgaris