Department of Dermatology, PD Hinduja Hospital and MRC, Veer Savarkar Marg, Mahim, Mumbai-400 016, India.
Indian J Dermatol Venereol Leprol. 2011 Mar-Apr;77(2):177-9. doi: 10.4103/0378-6323.77458.
We report a 38-year-old housewife with systemic Langerhans cell histiocytosis (LCH) presenting as a chronic vulvar and peri-anal ulcer. She had systemic involvement in the form of diabetes insipidus and bone "hot-spots". She responded favorably to etoposide, 6-mercaptopurine, and systemic steroids, and has been in remission since 10 years. Chronic vulvar ulcers not responding to routine therapy should not be neglected and need to be biopsied repeatedly to come to a specific diagnosis. The vulvar ulcer in our case provided a vital clue to a systemic LCH, with a successful outcome.
我们报告了一例 38 岁家庭主妇患有系统性朗格汉斯细胞组织细胞增生症（LCH），表现为慢性外阴和肛周溃疡。她有全身症状，包括尿崩症和骨骼“热点”。她对依托泊苷、6-巯基嘌呤和全身类固醇反应良好，自 10 年前以来一直处于缓解状态。对于常规治疗反应不佳的慢性外阴溃疡不应被忽视，需要反复活检以明确诊断。我们病例中的外阴溃疡为系统性 LCH 提供了重要线索，并取得了良好的结果。
