Näher H
Hautklinik, Ruprecht-Karls-Universität Heidelberg.
Hautarzt. 1993 Feb;44(2):99-102.
We report on a 45-year-old patient, who first developed pemphigus vegetans of the Neumann type 9 years ago and who presented with typical manifestations of the disease 6 years after the first exacerbation. On systemic therapy with corticosteroids progression of the disease ceased. Additional application of azathioprine, cyclophosphamide or gold thiomalate, however, did not make it possible to reduce the high corticosteroid dosage without exacerbation of the disease. The long-term corticosteroid therapy led to sepsis, thrombosis of the leg and pelvic veins and osteoporosis with crush fractures of the vertebrae; corticosteroid therapy was therefore discontinued and replaced by cyclophosphamide bolus therapy associated with gold thiomalate. The subsequent exacerbation of the pemphigus was treated with methotrexate and dapsone. The skin lesions progressed further, however, and the patient died of sepsis 39 weeks after admission to the clinic.
我们报告了一名45岁的患者，该患者9年前首次出现纽曼型增殖性天疱疮，首次病情加重6年后出现该病的典型表现。在接受全身性皮质类固醇治疗后，疾病进展停止。然而，额外应用硫唑嘌呤、环磷酰胺或硫代苹果酸金钠并不能在不加重病情的情况下降低高剂量的皮质类固醇。长期皮质类固醇治疗导致败血症、腿部和盆腔静脉血栓形成以及伴有椎体压缩性骨折的骨质疏松症；因此停止了皮质类固醇治疗，并代之以与硫代苹果酸金钠联合的环磷酰胺大剂量冲击疗法。随后天疱疮病情加重，采用甲氨蝶呤和氨苯砜治疗。然而，皮肤病变进一步发展，患者在入院39周后死于败血症。
