Twelve-year clinico-therapeutic experience in pemphigus: a retrospective study of 54 cases.

BACKGROUND: Pemphigus, a common immunobullous disease of skin and mucous membranes affecting both sexes of all ages, was almost fatal before the advent of corticosteroids. Better strategies to avoid their side-effects and recent introduction of adjuvant therapy has further improved its prognosis. As the treatment remains need-based and patient-specific, different regimens and strategies have evolved, each with its own merits and demerits. This retrospective hospital-based study was carried out to understand the clinico-therapeutic aspects of pemphigus in our clinic.
METHODS: Medical records of all new patients admitted to our hospital with the diagnosis of pemphigus from 1990 to 2002 were analyzed. The diagnosis was mainly clinical and confirmed by positive Tzanck's test and histopathology. All patients were assessed clinically on a severity score of 1+ to 4+. These patients had received treatment with dexamethasone-cyclophosphamide pulse (DCP) therapy, oral mini-pulse (OMP) with betamethasone, or intramuscular triamcinolone acetonide alone or with azathioprine, dapsone or cyclophosphamide. They were followed up for clinical remission and side-effects of therapy.
RESULTS: There were a total of 54 new patients comprising 53.7% females and 46.3% males, and 12.9% of these were < 18 years of age. Pemphigus vulgaris was the commonest clinical type seen in 81.48% and mucosal involvement was seen in 63.63% of cases. The severity of mucosal lesions was not proportionate to that of cutaneous lesions. Associated diseases seen were seropositive rheumatoid arthritis, hypertension, diabetes mellitus and hyperthyroidism in one case each. Dexamethasone-cyclophosphamide pulse therapy was given to 75% of the pemphigus vulgaris patients while those having less severe disease were treated with other regimens. In general, clinical remission was seen after 2-16 (mean 6.5) DCP doses. Two patients have been in complete remission for the last 5 and 7 years of completion of DCP therapy, respectively. Addition of other adjuvants to corticosteroids was also helpful. However, azathioprine 50 mg/day was not as effective as cyclophosphamide 50 mg/day. Menstrual irregularities, amenorrhoea, azoospermia, rise in blood pressure and glycosuria were the major side-effects seen during DCP pulse therapy. Drop out rate was unacceptably high with all modes of treatment, although with DCP therapy it appears to be partly owing to early disease control. There was no mortality in this series.
CONCLUSIONS: Pemphigus vulgaris is the commonest clinical type. Mucosal surfaces other than the oral cavity are uncommonly involved, it may herald the onset of disease and takes longer to heal. Dexamethasone-cyclophosphamide pulse therapy seems to have a definite advantage over treatment with steroids alone, especially in terms of better control of disease activity, near absence of steroid side-effects and significantly reduced hospital stay. However, ways and means to reduce gonadal toxicity of adjuvants need to be explored as DCP therapy is likely to stay as a treatment of choice.