中国麻风皮肤病杂志 ›› 2025, Vol. 41 ›› Issue (3): 191-197.doi: 10.12144/zgmfskin202503191

• 临床研究 • 上一篇    下一篇

以皮肤溃疡为主要表现的多系统朗格汉斯细胞组织细胞增生症一例并文献复习

刁晗1,吕雅琳1,臧莹2,王梓赫1,王君1   

  1. 1青岛大学附属医院皮肤科，山东青岛，266000； 2仪征市人民医院，江苏扬州，211400
  • 出版日期:2025-03-15 发布日期:2025-03-03

Multisystem Langerhans cell histiocytosis with skin ulcers as the main manifestation: a case report and literature review

DIAO Han1, LYU Yalin1, ZANG Ying2, WANG Zihe1, WANG Jun1   

  1. 1 The Affiliated Hospital of Qingdao University, Qingdao 266000, China; 2 Yizheng People's Hospital, Yangzhou 211400, China
  • Online:2025-03-15 Published:2025-03-03

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摘要： 朗格汉斯细胞组织细胞增生症是一种罕见的系统性疾病，骨骼是最常见的受累部位，本例患者，男，32岁，以皮肤溃疡为主要表现，累及肺、骨骼、血液等多系统，给予甲氨蝶呤联合阿糖胞苷治疗8个周期后，疗效良好，大部分皮损愈合。检索朗格汉斯细胞组织细胞增生症和靶向治疗相关的文章，通过Pubmed共检索到17篇，中文数据库共检索到5篇。共收集到28例患者病例资料，其中成人8例，儿童20例，皮肤（23例）和骨骼（12例）是常见的受累部位，靶向治疗对难治性或复发性患者有很好的疗效。

关键词: 成人, 朗格汉斯细胞组织细胞增生症, 皮肤溃疡, 靶向治疗

Abstract: Langerhans cell histiocytosis is a rare systemic disease, and the bone is the most commonly affected site. A 32-year-old male patient presented with skin ulcer as the main manifestation, involving lung, bone, blood and other systems. After 8 cycles of methotrexate combined with cytarabine, the patient had a good effect and most of the skin lesions healed. A total of 17 articles related to Langerhans cell histiocytosis and targeted therapy were retrieved from Pubmed and 5 articles were retrieved from Chinese databases. A total of 28 cases were collected, including 8 adults and 20 children. Skin (23 cases) and bone (12 cases) were common sites of involvement, and targeted therapy has a good effect on refractory or relapsed patients.

Key words: adult, Langerhans cell histiocytosis, skin ulcer, targeted therapy