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Journal of the American Academy of Dermatology
Volume 42, n° 3
pages 422-427 (mars 2000)
Doi : 10.1016/S0190-9622(00)90213-5
accepted : 7 July 1999
Patterns of remission in pemphigus vulgaris
 

Andrew Herbst, MD, Jean-Claude Bystryn, MD
Ronald O. Perelman Department of Dermatology, New York University Medical Center. New York, New York 

Abstract

Background: The incidence of remissions in pemphigus is unclear because these are usually reported at a single point in the evolution of the disease. Thus it is uncertain whether treatment simply suppresses the manifestations of the disease and consequently must be continuously administered, or induces complete and long-lasting remissions that permit therapy to be discontinued. Objective: To answer this question, we investigated the incidence of remission in a long-term longitudinal study. Methods: The induction of complete and long-lasting remissions (lesion free with no systemic therapy for at least 6 months) was studied in 40 patients with pemphigus vulgaris treated conventionally and followed up for an average of 7.7 years by the same investigator. Results: Five (5%) of the patients died of the disease. Complete and long-lasting remissions were induced in 25%, 50%, and 75% of patients 2, 5, and 10 years, respectively, after diagnosis. Most of the remaining patients were in partial remission or had mild disease controlled with a small dose of steroids. The course of the disease followed different patterns, with some patients rapidly entering complete and long-lasting remissions, whereas others never entered into a complete remission. The induction of complete remission was related to the initial severity and extent of disease and to early response to treatment. Conclusion: It is possible to eventually induce complete and durable remissions in most patients with pemphigus that permit systemic therapy to be safely discontinued without a flare in disease activity. The proportion of patients in whom this can be achieved increases steadily with time, and therapy can be discontinued in approximately 75% of patients after 10 years. (J Am Acad Dermatol 2000;42:422-7.)

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 Supported in part by New York University Skin Disease Research Center Grant No. 5 P30 AR39749.
 Reprint requests: Jean-Claude Bystryn, MD, Department of Dermatology, New York University School of Medicine, 560 First Ave, Room H-356, New York, NY 10016.



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