Sander C S, Kaatz M, Elsner P
Department of Dermatology and Allergology, Friedrich Schiller University Jena, Jena, Germany.
Dermatology. 2004;208(2):149-52. doi: 10.1159/000076491.
Langerhans cell histiocytosis (LCH) represents a group of rare histiocytic syndromes characterized by tissue infiltration with dendritic cells. The management of LCH is difficult as these disorders respond inconsistently to immunosuppressive and chemotherapeutic strategies. Thalidomide (N-phtalimidoglutarimide), initially used as a tranquilizer, has recently been used in the management of several inflammatory skin diseases. We describe the case of a 38-year-old male with mucocutaneous LCH. A treatment course with 6 cycles of 2-chlorodeoxyadenosin (cladribine) was initiated. This was well tolerated but withdrawn after 6 months to prevent secondary malignancy. A partial remission was seen. Subsequently, a treatment course with thalidomide 200 mg daily was started. This therapy resulted in a significant improvement of the mucocutaneous lesions within 4 weeks and complete healing was achieved after 3 months. Treatment was then successfully continued with daily doses of 100 mg to prevent relapse. In conclusion, thalidomide monotherapy represents an effective, safe and well-tolerated treatment option that should be considered as first-line therapy for mucocutaneous LCH.
朗格汉斯细胞组织细胞增多症（LCH）是一组罕见的组织细胞综合征，其特征是树突状细胞浸润组织。LCH的治疗很困难，因为这些疾病对免疫抑制和化疗策略的反应不一致。沙利度胺（N-邻苯二甲酰谷氨酸）最初用作镇静剂，最近已用于治疗几种炎症性皮肤病。我们描述了一名38岁男性黏膜皮肤LCH的病例。开始了一个使用2-氯脱氧腺苷（克拉屈滨）进行6个周期的治疗疗程。该治疗耐受性良好，但6个月后停药以预防继发性恶性肿瘤。可见部分缓解。随后，开始了一个每天服用200毫克沙利度胺的治疗疗程。该疗法在4周内使黏膜皮肤病变显著改善，3个月后实现完全愈合。然后成功继续每日服用100毫克以预防复发。总之，沙利度胺单药治疗是一种有效、安全且耐受性良好的治疗选择，应被视为黏膜皮肤LCH的一线治疗方法。
