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IgA Pemphigus — SCE Dermatology MCQ

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HardBlistering DiseasesIgA PemphigusSCE Dermatology

A 45-year-old woman presents with a generalised blistering eruption sparing the mucosae. She has subcorneal blisters that are flaccid and rupture easily. DIF shows intercellular IgA in the upper epidermis. What is the diagnosis?

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Correct answer: D — IgA pemphigus (subcorneal pustular dermatosis type)

The best answer is “IgA pemphigus (subcorneal pustular dermatosis type)”. The level of blister cleavage and the distribution of immunoreactants distinguish intraepidermal pemphigus-pattern disorders from subepidermal autoimmune blistering diseases. The alternatives “Pemphigus vulgaris, within an appropriate UK pathway”, “Linear IgA disease, after clinicopathological correlation”, “Dermatitis herpetiformis, after specialist assessment”, “Pemphigus foliaceus, when the full phenotype supports it” 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