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Indian Journal of Medical Sciences, Vol. 61, No. 3, March, 2007, pp. 126-127 Editorial A simple and effective approach to pemphigus Jonkman MarcelF Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen Code Number: ms07020 In this issue, Firooz et al. summarized the opinion of Iranian and other Asian experts on the treatment of pemphigus vulgaris (PV).[1] This chronic mucocutaneous bullous autoimmune disease has a relative high incidence in Asia. The experts that received the questionnaire had large experience in PV; the majority of them had treated between 100 and 1000 patients, of which most had seen 5-30 new patients in the last year. Histology with direct immunofluorescence microscopy was routinely used to confirm the diagnosis of PV. Serum analysis for anti-desmoglein antibodies by ELISA is not used; for monitoring disease remission, the direct Nikolsky's sign on intact skin is common practice. In contrast to American practice, most experts in Asia combine high dose prednisolone (1-2 mg/kg/day) with azathioprine at the initiation of PV treatment. Indeed, starting immediately with azathioprine allows complete tapering of prednisolone even within 19 weeks.[2] The dose of azathioprine is important and should be high enough-between 2 and 3 mg/kg/day. More expensive immunosuppressors like mycophenolate/mofetil and mycophenolic acid are rarely used in Asia as first line of treatment. These drugs, however, have a place in case of continued hepatotoxicity that may be caused by azathioprine. It is interesting to note that most Asian experts taper prednisolone completely, while proceeding with azathioprine for 1-2 years. Such a treatment policy is wise. The doctor and PV patient in Asia apparently prefer to accept the chance of limited PV relapse to the merit of decreased steroid-induced morbidity. Recently, we found by double-blind randomized controlled trial (PEMPULS) that adding dexamethasone pulse therapy to the above-mentioned standard combination regimen had no benefit in PV.[2] Of note is, that Iranian experts do not use dexamethasone-cyclophosphamide pulse therapy, as propagated by emeritus-professor Pasricha from New Delhi. The outcome of the PEMPULS trial supports their choice of treating PV by combination regimen only. New and expensive treatment options such as rituximab and intravenous immunoglobulin[3] should be reserved for those patients that are refractory for the standard combination regimen or those that have an absolute contraindication for the use of steroids. The need for common definitions and measurements for PV is recognized to allow future international multi-center clinical trials. For that an international Pemphigus Definitions Committee is currently active, led by Dr. Victoria P. Werth.[4] Asian dermatological experts may contribute to future clinical trials for this disease, which is more common in their continent. References
Copyright 2007 - Indian Journal of Medical Sciences |
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