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Dermatitis Herpetiformis — SCE Dermatology MCQ

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ModerateBlistering DiseasesDermatitis HerpetiformisSCE Dermatology

A 35-year-old woman with a history of coeliac disease presents with intensely pruritic, grouped vesicles on her elbows, knees, and buttocks. Biopsy from perilesional skin shows granular IgA deposits at the dermal papillae on direct immunofluorescence. What is the most appropriate long-term treatment?

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Correct answer: CStrict gluten-free diet and Dapsone

The correct answer is C, strict gluten-free diet and Dapsone. This patient's presentation, intensely pruritic grouped vesicles on extensor surfaces (elbows, knees, buttocks) in a coeliac patient, with granular IgA deposition at the dermal papillary tips on direct immunofluorescence, is diagnostic of dermatitis herpetiformis (DH), the cutaneous manifestation of gluten-sensitive enteropathy. Dapsone provides rapid suppression of neutrophil-mediated inflammation and controls the itch and blistering within days, but it does not address the underlying gluten-driven immune process. A strict lifelong gluten-free diet treats the enteropathy and, over months, allows the skin disease to remit, permitting dapsone to be tapered and often stopped; this combination is the recommended long-term strategy in UK dermatology guidance. Why the other options are wrong: A. Oral Ciclosporin: Not used in DH; ciclosporin has no established role in this IgA-mediated disease and carries nephrotoxicity risk without evidence of benefit. D. Topical clobetasol propionate alone: Potent topical steroids may give partial symptomatic relief but do not control the systemic gluten-driven process or prevent new lesion formation, and are not disease-modifying. E. Oral Prednisolone: Systemic steroids are generally ineffective in DH and are not recommended as they do not reliably suppress the granular IgA-driven neutrophilic response. B. Rituximab: Not indicated; DH is not a first-line rituximab-responsive disease, unlike some other IgA or autoimmune bullous disorders where B-cell depletion is used. Key point: In dermatitis herpetiformis, dapsone gives fast symptom control while a strict lifelong gluten-free diet is the only long-term disease-modifying treatment, allowing dapsone withdrawal over time.

Reference: British Association of Dermatologists guidelines for the management of dermatitis herpetiformis 2018 (Harman KE et al., Br J Dermatol); BAD patient information leaflet, Dermatitis herpetiformis (gluten sensitivity), www.bad.org.uk/pils/dermatitis-herpetiformis-gluten-sensitivity