口腔粘膜に初発した尋常性天疱瘡10例の臨床的検討

概要

論文の詳細を見る
We report our clinical experience with 10 cases of pemphigus vulgaris(PV)initially arising in the oral mucosa. The patients were 3 males and 7 females, with a mean age of 47.4 years. The patients were classified into a mucosal type(PV-M)group or a mucocutaneous type(PV-MC)group according to their serum levels of antidesmoglein(Dsg)-1 and Dsg-3 autoantibodies. Their clinical features and treatment outcomes were assessed.The most commonly affected sites in the oral cavity were the gingiva and buccal mucosa in both the PV-M and PV-MC groups. Skin involvement was frequent in the PV-MC group(80 %), but not in the PV-M group(40 %).Systemic steroid therapy was given to all of the patients, and both the oral and skin lesions completely disappeared within 1 month. Seven patients had relapses after tapering of the steroid dosage to the maintenance level.All patients with relapse had mucosal lesions in the oral cavity.We examined the serum levels of autoantibodies to Dsg-1 and Dsg-3 and evaluated the correlation between the serum autoantibody level and severity of disease. Patients with severe oral symptoms had higher serum levels of Dsg autoantibodies. However, the severity of the skin lesions was not strongly related to the serum autoantibody levels.