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Mucous Membrane Pemphigoid — SCE Dermatology MCQ

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HardBlistering DiseasesMucous Membrane PemphigoidSCE Dermatology

A patient with mucous membrane pemphigoid has progressive scarring of the conjunctivae with symblepharon formation. DIF from a perilesional mucosal biopsy shows linear IgG and C3 at the basement membrane zone. Serology shows antibodies to BP180 (NC16A domain) and laminin-332. What additional investigation is warranted given the laminin-332 antibodies?

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Correct answer: DMalignancy screening

The correct answer is D, malignancy screening. Anti-laminin-332 (anti-epiligrin) mucous membrane pemphigoid is a distinct immunological subset in which the autoantibody targets the basement membrane anchoring protein laminin-332 rather than BP180 alone, and this subset carries a well-documented paraneoplastic association with underlying solid organ malignancy. Current European guidance, which underpins UK practice, states that malignancies have been reported in a substantial proportion of MMP patients with anti-laminin 332 reactivity, so detection of this antibody should trigger a search for an occult tumour (commonly lung, stomach, colorectal or other solid organ cancers) alongside optimisation of immunosuppressive treatment to prevent blinding conjunctival scarring. The severity of the ocular disease (symblepharon) reinforces the need for aggressive systemic therapy, but the specific antibody profile is what drives the additional malignancy workup, not the ocular severity itself.

Reference: https://www.nice.org.uk/guidance/conditions-and-diseases/skin-conditions