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Darier Disease — SCE Dermatology MCQ

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HardBlistering DiseasesDarier DiseaseSCE Dermatology

A 55-year-old man has Darier disease and develops eczema herpeticum (Kaposi varicelliform eruption) superimposed on his Darier lesions. Why are patients with Darier disease particularly susceptible?

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Correct answer: CDarier disease causes defective epidermal barrier and impaired innate immunity due to disrupted calcium-dependent antimicrobial peptide release

The best answer is “Darier disease causes defective epidermal barrier and impaired innate immunity due to disrupted calcium-dependent antimicrobial peptide release”. Eczema treatment is selected by site and severity, with topical corticosteroid or calcineurin-inhibitor safety, infection, adherence, contact allergy and psychosocial effects considered before escalation. The alternatives “They use topical corticosteroids, within an appropriate UK pathway, after clinicopathological correlation, after specialist assessment”, “They have fungal co-infection, after clinicopathological correlation, after specialist assessment, when the full phenotype supports it”, “They have excessive UV exposure, after specialist assessment, when the full phenotype supports it, within an appropriate UK pathway”, “They are immunosuppressed, when the full phenotype supports it, within an appropriate UK pathway, after clinicopathological correlation” are clinically adjacent possibilities, but they do not fit the defining morphology, distribution, histopathology, risk signal or management sequence in this stem.

Reference: NICE CG57 atopic eczema recommendations: https://www.nice.org.uk/guidance/cg57/chapter/Recommendations