Langerhans' cell histiocytosis of the vulva: the Iowa experience.

Background: Vulvar presentation of Langerhans' cell histiocytosis (histiocytosis X) is rare. Symptoms and signs at the time of presentation can include pruritus, pain, dyspareunia, burning, discharge and presence of a discrete lesion and/or generalized ulceration. Once a diagnosis of Langerhans' cell histiocytosis is made, there is no formal treatment protocol. This report highlights 2 clinical cases diagnosed and treated at a tertiary care center.

Cases: We report the case histories of 2 women who presented to the Vulva/Vaginal Disease Clinic at the University of Iowa. The first patient, a 76-year-old woman, had a 1-year history of vulvar pruritus. The second patient, a 39-year-old woman, had a 3-month history of a clitoral growth.

Conclusion: Presentation of Langerhans' cell histiocytosis as a vulvar primary condition is rare and probably underdiagnosed as its clinical presentation can vary. Such patients often have been treated for recurrent yeast or presumed herpes simplex virus infections. Langerhans' cell histiocytosis should be considered as a differential diagnosis in females who present with chronic pruritus, pain, ulcerations or intermittent rashes.