Anti-p200 pemphigoid was first characterized in 1996, and less than 200 cases have been reported since its discovery. This rare autoimmune blistering disease is characterized by tense, pruritic subepidermal bullae distributed mainly over the hands and feet. Bullae may also involve the trunk, extremities, and mucous membranes (notably the oral and anogenital mucosa). It generally presents in patients in their sixth to eighth decade, which is a slightly younger patient population than that of bullous pemphigoid (BP).

Autoantibodies to a 200-kDa protein in the lamina lucida are responsible for the disease. Studies have tried to characterize this protein, but the protein remains unidentified at this time. Suspicion exists for involvement of anti-laminin γ1 antibodies in disease pathogenesis. However, no correlation has been found between anti-laminin γ1 antibodies and blister formation in ex vivo studies.

Some believe anti-p200 pemphigoid may follow a milder course than other autoimmune bullous disorders. However, more recently, a spectrum of disease severity has been recognized, with treatment-resistant cases having also been reported.