Supportive Data

Thirty-two classic bullous pemphigoid (BP), 15 mucous membrane pemphigoid, and 7 other pemphigoid variants, diagnosed by direct immunofluorescence, routine histology, and clinical presentation were tested. Controls included 47 patients with other autoimmune blistering disorders and 42 age-matched controls without skin disease. Forty of 54 (74%) patients with BP and variants tested positive for BP180 and/or BP230 autoantibodies. Of these patients, 28 of 32 (88%) with classical BP, 8 of 15 (53%) with mucous membrane predominant (MMP), and 4 of 7 (57%) of other pemphigoid variants, tested positive.

The calculated sensitivities in classical BP were 54% for BP180 alone and 56% for BP230 alone. The sensitivity increased to 88% with both tests combined, which is comparable to that of indirect immunofluorescence (IIF) (88%). In MMP the calculated sensitivities were 47% for BP180 alone, 13% for BP230 alone, and 53% for both combined. This was slightly less than the sensitivity of IIF (67%). Only 5 of 47 (11%) and 2 of 47 (4%) control patients with other autoimmune blistering disorders were positive for BP180 and BP230 autoantibodies respectively. Interestingly, the 2 patients positive for BP230 autoantibody had paraneoplastic pemphigus. One of 42 (2%) and 0 normal controls tested positive for BP180 and BP230 respectively.

The calculated specificities for BP180, BP230, and IIF were 93%, 98%, and 92% respectively.