High-dose intravenous 'pulse' methylprednisone in the treatment of severe oropharyngeal pemphigus: a pilot study.

BACKGROUND: High-dose intravenous (i.v.) methylprednisolone has been used therapeutically in severe blistering diseases to avoid the complications and side-effects of long-term orally administered glucocorticoid therapy. The aim of the study is to evaluate the capacity of methylprednisolone i.v. 'pulse' therapy to induce remission in the treatment of severe oropharyngeal pemphigus. METHODS: Twelve patients, all of whom had oropharyngeal severe pemphigus, were included in the analysis. There were eight women and four men whose ages ranged from 22 to 78 years (mean age: 50.75 years) with a disease duration of 1-3 months(mean duration: 55 days). In order to obtain a rapid clinical remission of extensive mucosal lesions, we performed 'pulse' therapy with intravenous methylprednisolone (30 mg/kg body weight to a maximum of 1 g per dose on each of 3-5 consecutive days)evaluating the clinical response and the short-term side-effects. RESULTS: Our therapy was generally safe and well tolerated with a very low rate of side-effects. All patients responded to i.v. methyl-prednisolone with evidence of a decrease in signs and symptoms within l week of commencing treatment and in all cases remission was observed after the second or the third cycle of 'pulse'. The most common adverse events during treatment were flushing and hyperglycaemia; in a few cases we observed a metallic taste in the mouth, pruritus, headaches ranging from mild to moderate, palpitations, mood alterations, insomnia and fatigue. CONCLUSIONS: High-dose 'pulse' administration of glucocorticoids is a potentially effective therapy to be considered in the treatment of patients with severe oropharyngeal pemphigus. Similar patients treated with conventional oral administered doses of prednisone or deflazacort had protracted courses requiring months of glucocorticoid therapy with no long-term remissions. However, further well-designed, long-term comparative trials are required to confirm this.