Mycosis fungoides of the vulva

Mycosis fungoides (MF) is an indolent form of non-Hodgkin lymphoma and the most common type of primary cutaneous T-cell lymphoma. The overall incidence of MF is approximately 4 per 1 million. Involvement of the vulva by MF is extremely rare, with only seven reported cases in the literature. At the vulva, it is mainly a metastatic lesion and rarely a primary malignancy. We describe a case of vulvar MF and discuss the previous cases. The presentation can easily be confused with benign skin disorders. A vulvar lesion can reflect a systemic disease. When a patient consults for a vulvar lesion it is therefore important not only to look at the vulva but also to examine her in and ask general questions. In a patient with a vulvar mass and cutaneous lesions on other locations MF should be considered in the differential diagnosis.