Vulvar Langerhans cell histiocytosis: a case report and review of the literature

Abstract

Background Langerhans cell histiocytosis (LCH) of the female genital tract is rare. Only, seven cases of primary vulvar LCH have been previously reported in the medical literature. We describe an additional case of LCH in which the disease was confined to the vulva. Case A 33-year-old gravida 0, para 0 Ethiopian woman presented with a nodular lesion on her left vulva. The lesion was biopsied, and the results were consistent with LCH. A metastatic workup did not reveal any evidence of disease beyond the vulva. The patient was initially treated with radiotherapy to the vulva. She was diagnosed with recurrent disease in the vulva 21 months after the completion of radiotherapy. At that time, she underwent a wide local excision. Five months later, we found a lesion on her right labium majus that was consistent with a recurrence. The patient's vulva was treated with a higher dose of radiotherapy than it had been the first time. Six months later the patient again experienced a local recurrence. She underwent a wide radical vulvar excision of diffuse bilateral lesions and was free of disease for approximately 3 months, after which she experienced another recurrence and underwent treatment with thalidomide. Within 2 months of starting thalidomide therapy, the patient experienced resolution of her symptoms and of her vulvar lesions. Conclusion Primary LCH of the vulva is very rare. Its etiology and pathophysiology, as well as the most effective modes of therapy, remain elusive. We propose that thalidomide is a useful alternative for patients with this disease.