Initial management of pemphigus vulgaris and pemphigus foliaceus

AZA: azathioprine; IVIG: intravenous immune globulin; MMF: mycophenolate mofetil.

* Rituximab given in conjunction with high-potency topical corticosteroids rather than prednisone is sometimes used as an alternative for mild disease. However, efficacy data are limited.

¶ Disease control is defined as the point at which there is cessation of new lesion formation and established lesions begin to heal.

Δ Examples of therapies reserved for refractory pemphigus include IVIG, cyclophosphamide, immunoadsorption, and plasmapheresis.

◊ Prednisone tapers are usually started after disease control has been stable for at least 2 weeks and 80% of established lesions have healed. Tapering is performed slowly. Refer to additional UpToDate content on the initial management of pemphigus for details.

§ For patients receiving prednisone plus MMF or AZA, tapering of MMF or AZA may begin if at least 3 months have passed after successful cessation of prednisone. When tapering, we reduce AZA by 50 mg every 8 weeks or reduce MMF by 500 mg every 8 weeks. Taper until treatment cessation or to the lowest dose necessary to maintain remission.

¥ Relapse is defined as the appearance of 3 or more new lesions within 1 month that do not heal within 1 week or the extension of established lesions. Refer to additional UpToDate content on the initial management of pemphigus for details on relapse treatment.