Outcome of pemphigus vulgaris

Pemphigus vulgaris can be divided into mucosal, mucocutaneous and cutaneous subtypes. A higher mortality rate has been shown with mucocutaneous involvement.

The aim of this retrospective study was to analyse the association of clinical subtypes of pemphigus vulgaris with remission rates.

One hundred twenty-eight patients with pemphigus vulgaris, treated with prednisolone 2 mg/kg/day plus azathioprine 2 to 2.5 mg/kg/day, were enrolled. The partial and complete remission rates, at the end of the first and second years of treatment, and the number of relapses were compared in the three groups.

After disease establishment, 71.1% had mucocutaneous, 18.8% had mucosal, and 10.2% had only cutaneous involvement. The mean duration of follow-up was 53.5 +/- 39.6 months. The mean duration it took the mucocutaneous group to reach a prednisolone dosage of 30 mg/day was significantly longer (P = 0.050). Mucocutaneous patients had a significantly lower rate of remission (31.9%) compared with those with only mucosal or cutaneous involvement (48.6%) at the end of the first year of the treatment (P = 0.029). After 2 years, mucocutaneous patients again had a lower remission rate (32.9% vs. 44.5%). Relapses were also more frequent in this subtype. Those presenting with mucosal or mucocutaneous erosions had a higher rate of active disease after receiving treatment for a year compared with those with only cutaneous presentation (66.7% vs. 45%; P = 0.057).

In mucocutaneous subtype, clinical control was achieved later, and they had a lower rate of remission at the end of the first and second years of treatment. They were also prone to relapses.