Diagnosis is confirmed by biopsy of perilesional skin, which reveals many eosinophils and neutrophils in and below the bulla at the dermoepidermal junction. On electron microscopy, the split is seen at the level of the lamina lucida where the bullous pemphigoid antigen is found. Control is usually achieved with a daily dose of 60 to 80 mg of prednisone initially, followed by fairly rapid tapering to 30 to 40 mg and the slower tapering. Dapsone, cyclophosphamide, or azathioprine is often added if there is difficulty tapering the steroid. The disease often remits in 1 to 2 years, and steroids can be stopped.
Lesions begin around or in the mouth, or on the scalp, and can spread to any area.
Primary lesions are flaccid, noninflamed bullae, which break easily and leave large denuded areas, which then crust. Lesions heal with temporary hyperpigmentation but without scarring. Therapy is with high-dose steroids, which dramatically reduces the mortality, and referral to a dermatologist is indicated. If the dose nears 200 mg, consider IV pulse therapy with steroids or plasmapheresis. A vascular reaction with a wide variety of causes.