Treatment of Pemphigus Vulgaris and Pemphigus Foliaceus: Experience with 30 Patients

N. Fernandes
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Abstract

Pemphigus vulgaris and Pemphigus foliaceus are chronic, painful diseases which treatment causes great impact. This study focuses on the age, gender, mucosal lesions, therapy with oral prednisone, clinical remission rates and side effects. Material And Methods : Six males/15 females with Pemphigus vulgaris and three males/six females with non-endemic pemphigus foliaceus attended at Hospital Universitario from 2003 to 2018 were submitted to histopathology through skin or mucosa biopsy. Oral prednisone (monotherapy) in seven Pemphigus vulgaris and two pemphigus foliaceus; in case of no control or relapses, association with cyclophosphamide pulse therapy (12PV/4PF), dexamethasone pulse therapy (ten PV/two PF), hydroxychloroquine (three PV/four PF), azathioprine (five PV),methotrexate (four PV/one PF), immunoglobulin cycles (two PV/two PF). Results : Mostly patients were female aged 30-59 years old; oral mucosal lesions in 19 (90,4%) pemphigus vulgaris. After a ten year period of monitoring it was observed in the group of monotherapy: one PV/one PF achieved complete remission on low dose of prednisone; two PV achieved partial remission on low dose and four PV/one PF on high dose. In the group with combined drugs, four PV were off prednisone, four PV/one PF achieved complete remission on low dose and two PF on high dose; two PV/three PF achieved partial remission on low dose and four PV/one PF on high dose. The most common side effects of prednisone were mucocutaneous candidiasis, arterial hypertension, subcapsular posterior cataracts, bacterial and viral infections and diabetes mellitus. Conclusion : Both diseases ran a chronic and unpredictable course which management was extremely difficult. Females with pemphigus vulgaris developed severe multiple mucosal lesions.
寻常型天疱疮和叶状天疱疮30例的治疗体会
寻常型天疱疮和叶状天疱疮是一种慢性、疼痛性疾病,治疗效果显著。本研究的重点是年龄,性别,粘膜病变,口服强的松治疗,临床缓解率和副作用。材料与方法:2003 - 2018年在Universitario医院就诊的6例寻常型天疱疮患者(男15例)和3例非地方性叶状天疱疮患者(男6例)通过皮肤或粘膜活检进行组织病理学检查。口服强的松单药治疗7例寻常型天疱疮和2例叶状天疱疮在没有控制或复发的情况下,与环磷酰胺脉冲治疗(12PV/4PF)、地塞米松脉冲治疗(10pv / 2pf)、羟氯喹(3pv /4PF)、硫唑嘌呤(5pv)、甲氨蝶呤(4pv / 1pf)、免疫球蛋白周期(2pv / 2pf)相关。结果:患者以女性为主,年龄30 ~ 59岁;寻常型天疱疮口腔黏膜病变19例(90,4%)。经过10年的监测,在单药治疗组中观察到:1名PV/ 1名PF在低剂量强的松治疗下完全缓解;低剂量组2例PV部分缓解,高剂量组4例PV/ 1例PF缓解。联合用药组,4例PV停药,低剂量组4例PV/ 1例PF完全缓解,高剂量组2例PF完全缓解;低剂量组2个PV/ 3个PF部分缓解,高剂量组4个PV/ 1个PF部分缓解。强的松最常见的副作用是皮肤粘膜念珠菌病、动脉高血压、后囊膜下白内障、细菌和病毒感染以及糖尿病。结论:两种疾病病程均为慢性且难以预测,治疗极为困难。寻常型天疱疮女性患者出现严重的多发性粘膜病变。
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