title: Pure cutaneous histiocytosis X of the vulva.
abstract: 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.
mesh_terms: D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000473:pathology* / Q000532:radiotherapy; D006801:Humans; D008875:Middle Aged; D011879:Radiotherapy Dosage; D012871:Skin Diseases / Q000175:diagnosis / Q000473:pathology* / Q000532:radiotherapy; D014845:Vulvar Diseases / Q000175:diagnosis / Q000473:pathology* / Q000532:radiotherapy