Verma Kaushal K, Srivastava P, Minz Anil, Verma Kamna
Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India.
Lepr Rev. 2006 Sep;77(3):225-9.
The pathogenesis of erythema nodosum leprosum (ENL) involves both immune complex deposition and dysfunction of cell mediated immunity. Tumour necrosis factor-alpha (TNF-alpha) plays an important role in its pathogenesis. Thalidomide and corticosteroids are the mainstay of treatment for ENL. However, there are often severe limitations to their use. We report a case of recurrent ENL treated successfully with azathioprine. A 15-year-old unmarried girl with lepromatous leprosy had recurrent ENL for 2 years. She was treated with WHO-MB MDT and prednisolone in doses of 40-90 mg a day for 2-12 weeks. Her condition was inadequately controlled. The patient was therefore treated with thalidomide 300 mg and prednisolone 40 mg. The symptoms subsided after 5 days and ENL lesions healed in 2 weeks. Prednisolone was reduced by 10 mg per week and stopped, while thalidomide was reduced to 100 twice daily after 4 weeks. Azathioprine 100 mg (2 mg/kg per day) daily orally was added to prevent recurrences. Thalidomide was further reduced and stopped after another 4 weeks while she continued with azathioprine in the same doses for 8 months. There was no recurrence of ENL lesions and no side effects of the therapy. MB-MDT was stopped 1 year ago, and she is on follow-up without any relapse. Azathioprine, therefore, appears to be an effective and safe drug to prevent recurrences of ENL.
结节性麻风红斑(ENL)的发病机制涉及免疫复合物沉积和细胞介导的免疫功能障碍。肿瘤坏死因子-α(TNF-α)在其发病机制中起重要作用。沙利度胺和皮质类固醇是ENL治疗的主要药物。然而,它们的使用往往存在严重局限性。我们报告一例用硫唑嘌呤成功治疗复发性ENL的病例。一名15岁未婚的瘤型麻风女孩复发性ENL已2年。她接受了世界卫生组织多菌型麻风联合化疗(WHO-MB MDT),并每日服用40-90毫克泼尼松龙,持续2-12周。她的病情控制不佳。因此,该患者接受了300毫克沙利度胺和40毫克泼尼松龙治疗。5天后症状消退,ENL皮损在2周内愈合。泼尼松龙每周减量10毫克并停用,而沙利度胺在4周后减至每日两次,每次100毫克。加用每日口服100毫克(2毫克/千克/天)硫唑嘌呤以预防复发。又过了4周,沙利度胺进一步减量并停用,而她继续以相同剂量服用硫唑嘌呤8个月。ENL皮损未复发,治疗也无副作用。1年前停用了MB-MDT,她正在接受随访,无任何复发情况。因此,硫唑嘌呤似乎是预防ENL复发的一种有效且安全的药物。