The treatment of pemphigus vulgaris. Experience with 48 patients seen over an 11-year period

Abstract

We present a retrospective analysis of 48 patients with pemphigus vulgaris (PV) who were seen between 1978 and 1988. They were divided into three treatment groups: 25 (group A) received 40-100 mg of oral prednisone daily; eight (group B) received > 100 mg of prednisone daily; 15 (group C) received 40 mg of oral prednisone every other day and azathioprine 100 mg daily. A second immunosuppressive agent was subsequently added to the treatment regimen of three patients in group A and eight patients in group B.By 1989. 10% of the patients had been able to discontinue all therapy, and were in complete remission. Sixty-five per cent of patients were on maintenance therapy, but in clinical remission. Twenty-five per cent of the patients had died [eight in group A (31%) and four in group B (50%)] either as a consequence of the disease or its treatment. None of the patients in group C had died. Most of the deaths occurred during the first 2-3 months of therapy. Morbidity and mortafity were related to the severity of the disease, to the maximum dose of prednisone required to induce remission, and to the presence of other diseases. Patients needing a total of 5 g or more of prednisone to induce a remission during the acute stage had a high mortality rate.

Management of oral pemphigus vulgaris: A case report and a clinical update

“…Prednisolone (1 mg/kg/ day), with or without other immunosuppressive agents, should be initiated immediately. 12,13 It should be continued until there is cessation of new bullae formation and Nikolsky's sign can no longer be elicited. The dosage is then reduced by one half until all of the lesions have cleared, followed by tapering to a minimum effective maintenance dosage.…”

Epidemiologic survey of pemphigus vulgaris with oral manifestations in northern Greece: Retrospective study of 129 patients

“…According to various authors, the onset and course of PV may be the result of an interaction between the host's genetic factors and environmental triggering agents 10,18,20–23 . Treatment initially includes the administration of systemic corticosteroids and other anti‐inflammatory and immunosuppressive drugs in order to reduce steroid dosage 24–28 . Epidemiologic data on PV are available in many countries, 29–46,49 while in northern Greece limited data are available on this field 26,29 .…”

Value of direct immunofluorescence in predicting remission in pemphigus vulgaris

“…A difficult management decision is how to maintain control with the least medication. Long‐term remission off all therapy has been reported to occur in 10–75% of the patients 7,8 . The differentiation between drug‐induced clinical control of the disease in the presence of autoantibodies, from immunological inactivity can help us to detect the subgroup of patients who maintain clinical remission after discontinuation of treatment.…”