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