Alioua Z, Hjira N, Oumakhir S, Frikh R, Ghfir M, Rimani M, Sedrati O
Service de dermatologie, hôpital militaire d'instruction Mohammed-V, BP 1018, Rabat, Maroc.
Rev Med Interne. 2006 Aug;27(8):633-6. doi: 10.1016/j.revmed.2006.01.022. Epub 2006 Mar 24.
Adult multisystem Langerhans cell histiocytosis is an excepted disorder, which have several treatments. The purpose of this study was to test the disease-controlling effect of thalidomide in a case of adult multisystem Langerhans cell histiocytosis with cutaneomucous and hypothalamic localizations at dermatology department, of Mohammed-V military hospital, Rabat.
A 43-year-old women, presented multifocal chronic Langerhans cell histiocytosis confined to cutaneous, oral cavity, perianal, mastoid and hypothalamic areas, with severe disabling ulcers in intertriginous areas, diabetes insipid and amenorrhoea. We treated with thalidomide 200 mg/day after neurological examination. A rapid initial response with total diminution of the involved skin area, and diminution of diabetes insipid. She remained in remission for 1 year. No adverse effects from treatment were observed at clinical and electrophysiological examinations.
Thalidomide treatment is an adequate therapeutic measure in adult Langerhans cell histiocytosis, which is rare and difficult to treat. Our case showed the efficacy of thalidomide at cutaneomucosal and hypothalamic manifestations.
成人多系统朗格汉斯细胞组织细胞增多症是一种罕见的疾病，有多种治疗方法。本研究的目的是在拉巴特穆罕默德五世军事医院皮肤科，对一例皮肤黏膜和下丘脑受累的成人多系统朗格汉斯细胞组织细胞增多症患者，测试沙利度胺的疾病控制效果。
一名43岁女性，患有多灶性慢性朗格汉斯细胞组织细胞增多症，病变局限于皮肤、口腔、肛周、乳突和下丘脑区域，间擦部位有严重致残性溃疡，伴有尿崩症和闭经。经神经科检查后，我们给予沙利度胺200毫克/天进行治疗。治疗初期迅速见效，受累皮肤面积完全缩小，尿崩症症状减轻。她维持缓解状态1年。临床和电生理检查未观察到治疗的不良反应。
沙利度胺治疗是成人朗格汉斯细胞组织细胞增多症的一种合适治疗措施，该病罕见且难以治疗。我们的病例显示了沙利度胺对皮肤黏膜和下丘脑表现的疗效。
