Dexamethasone-cyclophosphamide pulse therapy for pemphigus.

BACKGROUND: During the last 12 years, we have used a different approach, arbitrarily designed by us, for treating pemphigus patients that has given us very different and encouraging results.

METHOD: The treatment schedule consists of giving 100 mg dexamethasone on 3 consecutive days and 500 mg cyclophosphamide on one day and repeating these pulses (DCPS) every 4 weeks. In between the DCPS, the patient receives only 50 mg cyclophosphamide orally daily and generally no corticosteroids. An essential component of the regimen is to administer a specified amount of the treatment for 1.5 years after achieving clinical remission.

RESULTS: Of the 300 patients enrolled for this treatment, 61 patients could not complete the treatment, whereas 12 patients have died, some of them due to unrelated causes. Of the remaining 227 patients, 190 patients (84%) have already completed the treatment and are free of the disease even after complete withdrawal of all treatment, the duration of posttreatment follow-up being more than 5 years in 48 patients, 2 to 5 years in 75 patients, and less than 2 years in 67 patients. The maximum duration of posttreatment follow-up is 9 years. The remaining patients are also showing the same trend. Twenty-four patients are in remission but have not yet completed the treatment schedule, whereas 13 patients are still having evidence of clinically active disease, although it has already become much milder. The blood levels of intercellular antibody also decrease as the treatment progresses. The side effects commonly observed during treatment with corticosteroids are generally absent or insignificant. The relapses of the disease, seen so far in 59 patients, have been observed mostly in those patients who defaulted during the treatment, but a further course of the DCP regimen led again to complete recovery.

CONCLUSIONS: If substantiated by further follow-up, this treatment schedule may prove curative in this potentially fatal disease.