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. Herpes simplex infection causes most mild, recurrent cases. More severe, widespread cases may be caused by other infectious agents, especially Mycoplasma species and viruses, and by medications, especially penicillins, sulfonamides, anticonvulsants, nonsteroidal anti-inflammatory drugs, and allopurinol.