Histiocytosis X of the vulva: a case report and review of the literature.
Histiocytosis X of the female genital tract is unusual. Thirty-two cases have been reported to date in the world literature. An additional case is reported herein, presenting as a vulvar ulcer in a 2.5-year-old child with osteolytic lesions of the skull, splenomegaly, and otitis media. The diagnosis of histiocytosis X may be established by identifying the Langerhans histiocyte, characterized by nuclear grooves, immunoreactivity for S-100 protein, and pentalamellar cytoplasmic structures seen by electron microscopy. Prognosis is difficult to determine with certainty. However, age of less than 2 years at presentation, multi-organ involvement, and/or organ dysfunction appear to be associated with a less favorable prognosis. The patient presented herein is currently receiving vinblastine chemotherapy for recurrence of disease, manifested as an osteolytic lesion in the skull.[1]References
- Histiocytosis X of the vulva: a case report and review of the literature. Otis, C.N., Fischer, R.A., Johnson, N., Kelleher, J.F., Powell, J.L. Obstetrics and gynecology. (1990) [Pubmed]
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