Pure vulvar Langerhans cell histiocytosis: a case report and literature review.

Langerhans cell histiocytosis (LCH) of the female genital tract is a very rare disease. Only 16 cases of primary vulvar LCH without subsequent systemic spread of disease have previously been published in the literature. We describe an additional case of LCH in which the lesion was confined to the vulva. A 52-year-old Caucasian woman presented for further investigation with a 6-month history of vulvar pruritus. Physical examination revealed multiple fine red papules on both labia minor. A metastatic workup did not reveal any evidence of disease beyond the vulva. The lesion was biopsied, and histological findings were characteristic of LCH. The patient was treated by local extirpation of both labia minor. Ten months after surgery, the patient has no signs of local recurrence or systemic spread. It is necessary to perform a biopsy of the lesions when a woman presents atypical chronic lesions on the vulva. Although different treatment has been proposed, complete surgical excision is fundamental in "pure" genital Langerhans cell histiocytosis as initial therapy.