Background: As a result of a lack of large-scale controlled studies, the diagnosis and management of pemphigus vulgaris (PV) has been solely on the basis of expert opinion, rather than on empirical evidence. We have completed a survey of worldwide experts on the diagnostic and therapeutic approaches to PV. Methods: We conducted a telephone-based survey of 24 physicians from academic, tertiary care centers worldwide with an average of 20 years experience treating pemphigus. Survey questions included referral patterns, diagnostic techniques, and therapeutic regimens. Results: Of those surveyed, 50% receive referrals within 6 months after onset of symptoms, 17% within 1 year, and 8% within 3 years. Diagnosis is secured by 96% using skin biopsy specimen with direct immunofluorescence, and by indirect immunofluorescence alone for 4%. None of the participating physicians make the diagnosis of PV solely on clinical and histologic evidence. Of the physicians, 75% initially treat with prednisone and 25% use other agents or attempt to eliminate potential triggers. The physicians who initially used noncorticosteroid drugs did so with no relation to the nature or extent of the disease. Of those surveyed, 50% use prednisone doses of 1 mg/kg/d, 31% use 1 to 1.5 mg/kg/d, and 19% use 1.5 to 3 mg/kg/d. Azathioprine is used as an adjuvant by 44%, mycophenolate mofetil by 20%, cyclophosphamide by 16%, and methotrexate by 8%. Complete discontinuation of prednisone was the goal for 37% whereas others were satisfied with doses from 2.5 to 10 mg/d. Conclusion: Wide variation exists in diagnostic techniques and treatment of PV, even among the world's experts. The lag time from symptom onset to referral emphasizes the need for heightened awareness. There is clearly a need for consensus standards with regard to patient stratification and randomized controlled trials.
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