[Histiocytosis X (Langerhans cell granulomatosis) of the vulva]

Isolated histiocytosis X (Langerhans cell granulomatosis) of the vulva is extremely rare. In most reported cases, vulval disease is associated with multitopic organ involvement. We report here on the second case in the world of isolated histiocytosis X of the vulva. The diagnosis could be established by immunohistochemical demonstration of anti-S 100 protein + Langerhans cells. The patient received a local radiation therapy of 10 gy and responded excellently.