By Gary M. White, MD
Anti-p200 pemphigoid (APP), aka anti-laminin γ1 pemphigoid, is a rare autoimmune bullous dermatosis that closely resembles classic bullous pemphigoid, but has tense blisters with preference for the hands and feet.
- About half of patients have oral involvement.
- Key features include subepidermal blisters, negative anti-BP180 and Type VII collagen antibodies and positive anti-laminin γ1 autoantibodies.
- Antibodies are found against a 200-kDa protein, localized within the lower lamina lucida of the basement membrane one. These antibodies seem to attack the laminin γ1-chain.
- The clinical appearance may resemble dyshidrosiform pemphigoid and epidermolysis bullosa acquisita (EBA). In contrast to EBA, APP patients do not have scarring or milia formation.
Almost all patients experience bulla and pruritus. Bulla are almost always found on the hands, with variable involvement of the arms, feet, and trunk.
Treatment usually involves topical and systemic steroids initially. Azathioprine, dapsone, and doxycycline may also be given.
This article contains an excellent review on diagnosing APP and differentiating it from classic bullous pemphigoid, EBA, and mucous membrane pemphigoid. JAMA Derm 2016;152;897.
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