Langerhans' cell histiocytosis on the vulva

Langerhans' cell histiocytosis (LCH) affecting the vulva alone is rare. At present, there are 51 published cases of LCH affecting the genital area. Of these, only 7 had LCH located in the genital area alone; in these cases, there was no subsequent systemic spread of the disease. A report of the 8th such case is presented with a review of the previous 7 cases.

A 40-year-old white woman presented with a several-month history of pruritic and ulcerous lesions on the vagina which had then spread to the vulva. No tiredness, general malaise, or associated temperature was observed. Physical examination disclosed an erythematous plaque on the labia minora, which was bilateral and slightly infiltrated. The rest of the physical examination brought no other disorders of interest to light. Histological findings were characteristic of LCH. Four cycles of iv vincristine (2 mg/m cycle) 15 days apart did not obtain objective or symptomatic improvement in the vulvar lesions. Therefore, a local and partial extirpation of both labia minora was performed. Eighteen months after surgery, the patient has no symptoms or signs of local recurrence or systemic spread.

Although the occurrence of LCH on the vulva is very unusual, we must bear this possibility in mind when a woman presents atypical chronic lesions on the genital mucosa. In such cases, it is necessary to perform a biopsy on the mucosa, rule out the possibility of systemic disease, and review the patient periodically in order to forestall a possible spread of the disease at any time.