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.
Otis
C N
CN
Department of Pathology, Baystate Medical Center, Springfield, Massachusetts.
Fischer
R A
RA
Johnson
N
N
Kelleher
J F
JF
Powell
J L
JL
D001847:Bone Diseases / Q000473:pathology; D002675:Child, Preschool; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D006801:Humans; D014845:Vulvar Diseases / Q000175:diagnosis / Q000473:pathology / Q000628:therapy