[Immediate treatment of bullous pemphigus with a corticosteroid-cyclophosphamide combination]

Optimal management of the patient with generalized bullous pemphigoid (BP) should both control the disease rapidly to avoid fluid losses and superinfection, and have minimal side-effects. Although their initial dosage is subject to some controversy, corticosteroids (CS) provide currently the basis of therapy. Immunosuppressants have been advocated to decrease CS dosages more rapidly and to reduce potential hazards of long-term systemic steroid therapy, because of their sparing effect on cumulated CS doses. They have a delayed activity ranging from three to six weeks after beginning of therapy. Chloraminophene has been used in most previous studies. The aim of this study was to assess retrospectively a combined CS-cyclophosphamide (CP) regimen. Ten patients with generalized BP were treated. In nine, dexamethasone sodium phosphate was given intravenously (mean: 1.56 mg/kg/day in prednisone equivalents; range: 1 to 1.90 mg/kg/day) at onset of treatment. Prednisolone was given orally when complete control--absence of any new lesion--was achieved. CP was given d'emblée at a daily dosage of 100 mg for at least 6 months when feasible (mean: 4 months; range 9 days to 15 months). Patients characteristics are summarized in table I. Results are shown in tables II and III. In brief, three deaths occurred following major side-effects of the drugs during the first three months of treatment. Further non lethal untoward effects included bone marrow toxicity due to CP and septicemia (4 patients). At the end of follow-up, 5 patients were in remission without treatment for 22 +/- 6 months and one was controlled with prednisolone alone.(ABSTRACT TRUNCATED AT 250 WORDS)