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Darier Disease — SCE Dermatology MCQ

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ModerateBlistering DiseasesDarier DiseaseSCE Dermatology

A 30-year-old man presents with recurrent crops of discrete keratotic brown papules on his trunk that worsen with heat and sweating. The lesions are mildly pruritic. He has longitudinal white and red nail bands with V-nicking. Biopsy shows acantholytic dyskeratosis with corps ronds and grains. What is the diagnosis?

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Correct answer: C — Darier disease

The combination of keratotic papules in a seborrhoeic distribution worsening with heat/sweating, characteristic nail changes (longitudinal white and red alternating bands with V-shaped notching), and histological findings of acantholytic dyskeratosis with corps ronds and grains is diagnostic of Darier disease (keratosis follicularis). Grover disease shares similar histology (acantholytic dyskeratosis) but is transient, occurs in older men, and lacks the nail changes. Hailey-Hailey shows full-thickness 'dilapidated brick wall' acantholysis without dyskeratosis. Darier disease is autosomal dominant (ATP2A2 mutations).

Reference: BAD 2019 Darier Disease Guidelines