Patterns of remission in pemphigus vulgaris

Background: The incidence of remissions in pemphigus is unclear because the se 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 indu ces complete and long-lasting remissions that permit therapy to be disconti nued. Objective: To answer this question, we investigated the incidence of remiss ion 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 aver age of 7.7 years by the same investigator. Results: Five (5%) of the patients died of the disease. Complete and long-l asting remissions were induced in 25%, 50%, and 75% of patients 2, 5, and 1 0 years, respectively, after diagnosis. Most of the remaining patients were in partial remission or had mild disease controlled with a small dose of s teroids. The course of the disease followed different patterns, with some p atients rapidly entering complete and long-lasting remissions, whereas othe rs never entered into a complete remission. The induction of complete remis sion was related to the initial severity and extent of disease and to ear l y response to treatment. Conclusion: It is possible to eventually induce complete and durable remiss ions in most patients with pemphigus that permit systemic therapy to be saf ely discontinued without a flare in disease activity. The proportion of pat ients in whom this can be achieved increases steadily with time, and therap y can be discontinued in approximately 75% of patients after 10 years.