Lair G, Marie I, Cailleux N, Blot E, Boullié M C, Courville P, Lauret P, Lévesque H, Courtois H
Département de médecine interne, CHU Rouen-Boisguillaume, Rouen, France.
Rev Med Interne. 1998 Mar;19(3):196-8. doi: 10.1016/s0248-8663(97)80720-5.
Langerhans cell histiocytosis is a rare disorder with a wide spectrum of diseases and various clinical behaviours. The prognosis depends on the patient's age at onset and the extension of the disease. Treatment is function of evolution and extension of the disease.
We report one case of Langerhans cell histiocytosis in a 33-year-old woman with resistant vulvar involvement whose cutaneous lesions improved with thalidomide. Complete healing of the genital lesions occurred within 1 month, but the patient discontinued her treatment after 3 months because of somnolence. Recurrence of the symptoms was observed within 2 weeks without treatment, but complete control was obtained with reintroduction of thalidomide. No severe adverse effect was detected at electrophysiological and clinical examinations.
The simplicity and the rapid effect of thalidomide on cutaneous lesions suggest that new clinical evaluation of this old drug that can also be used in some systemic disease might be of the value.
朗格汉斯细胞组织细胞增多症是一种罕见疾病，具有广泛的疾病谱和多种临床行为。预后取决于患者发病时的年龄以及疾病的累及范围。治疗取决于疾病的进展和累及范围。
我们报告一例33岁女性朗格汉斯细胞组织细胞增多症，其外阴受累且难以治愈，皮肤病变经沙利度胺治疗后改善。生殖器病变在1个月内完全愈合，但患者在3个月后因嗜睡而停止治疗。未治疗的情况下，2周内症状复发，但重新使用沙利度胺后症状得到完全控制。电生理和临床检查未发现严重不良反应。
沙利度胺对皮肤病变的治疗简单且起效迅速，这表明对这种可用于某些全身性疾病的老药进行新的临床评估可能具有价值。
