Pure cutaneous histiocytosis X of the vulva

Histiocytosis X of the vulva is extremely rare. In most reported cases, vulvar disease is associated with multiple organ involvement and systemic disease manifestations aiding in the diagnosis. Reported is a case of histiocytosis X presenting solely with vulvar lesions for eight years' duration. The diagnosis, pathology, and subsequent management are discussed. The literature of histiocytosis X of the vulva is reviewed, and its presentations and treatments summarized. Vulvar lesions unresponsive to therapy warrant biopsy and other causes of histiocytic reactions must be excluded to establish the diagnosis.