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Darier Disease — SCE Dermatology MCQ

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HardBlistering DiseasesDarier DiseaseSCE Dermatology

A patient with Darier disease has widespread malodorous hyperkeratotic papules despite trigger avoidance, emollients and appropriate topical therapy. There is no pregnancy potential or retinoid contraindication. Which escalation is most rational?

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Correct answer: CConsider oral acitretin or isotretinoin with specialist retinoid monitoring after topical failure

Explanation lettering: D = shown as A · E = shown as C · C = shown as D · A = shown as E

E is correct. Severe or extensive Darier disease that remains troublesome after topical measures may be treated with an oral retinoid such as acitretin or isotretinoin under specialist supervision. Pregnancy prevention, liver and lipid monitoring, mucocutaneous adverse effects and interactions must be addressed. Heat, sweat, friction, UV exposure and infection can worsen disease, but malodour alone does not justify indefinite antibiotics. Darier disease is an ATP2A2-related acantholytic dyskeratosis, not an autoimmune blistering or IgE-mediated disorder. Secondary infection is treated when clinically supported.

Reference: British Association of Dermatologists: Darier disease: https://cdn.bad.org.uk/uploads/2021/11/19174112/Darier-disease-PIL-Dec-2020.pdf