Langerhans Cell Histiocytosis on the Vulva: A Case Report and Review of the Literature

Corresponding author: Wen LI; Tel: +86-13601858540; Fax: +86-21-63520020; E-mail: [email protected] Langerhans cell histiocytosis (LCH), also known as Histiocytosis X, is characterized by an organ-specific infiltration of cells with many morphological features and immunohistochemical markers of Langerhans cells. Clinically, LCH ranges from self-healing lesions to a multisystem involvement with organ dysfunction resistant to current therapies. The lesions appear in multiple organs, for example in the bones, skin, and lungs. But genital LCH as the only manifestation of this disease is very unusual. Initially, Lane et al. reported LCH in a 6year-old child in 1939. We here report an additional case of LCH on the vulva. Langerhans cell histiocytosis (LCH) of the female genital tract is rare. Only 20 cases of primary vulva LCH have been previously reported in the medical literature. In this report, we describe an additional case of LCH on the vulva. A 28-year-old Chinese woman presented with a two-year history of ulcerous lesions on the left vulva. No associated temperature, tiredness, or general malaise was observed. A diagnosis of LCH had been made by biopsy. Histological and immunohistochemical findings were characteristic of LCH. After two weeks of combined medical therapy, including interferon, prednisone, and methotrexate (MTX), the lesion started to cicatrize. Now, the patient was well throughout a 18-month follow up, showing no symptoms or signs of local recurrence or systemic spread. The occurrence of LCH on the vulva is very unusual. It is necessary to perform a biopsy on the lesion, rule out the possibility of multiorgan involvement. There are no standard treatment options for this rare disease. The most effective treatment options remain elusive. In our case, combined medical therapy was proved to be effective.