Key points

• In most patients pemphigus can be controlled with a daily dose of prednisolone <100 mg/day. A typical dosage for a patient with moderate pemphigus is prednisolone 1 mg/kg/day. If there is no response in 5-7 days, the dosage is increased in increments of 0-25-0-5 mg/kg/day until blistering ceases, complications arise or a dosage of 2-0-2-5 mg/kg/day is reached. These dosages come from experience in large series; relevant RCTs have not been done.

• The addition of an immunosuppressant (azathioprine, cyclophosphamide, metho-trexate, ciclosporin or mycophenolate mofetil) to prednisolone has not been shown to provide additional benefit, but does cause additional adverse effects. RCTs are needed to prove the benefit of adding immunosuppressant therapy.

• In patients with severe widespread pemphigus vulgaris not responding to oral prednisolone, intravenous pulse corticosteroid may help to bring the disease under control, but there is no reduction in mortality. Data on the value of intravenous pulse corticosteroid are scanty.

• Immunomodulatory therapy with plasmapheresis or IVIG may be useful for controlling severe or recalcitrant disease, but whether the results are better than with corticosteroid treatment alone remains unknown, and requires RCTs.

• A large RCT is testing whether dapsone can reduce symptoms and induce remission in mild and moderate pemphigus.

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