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.
Department of Pathology, Baystate Medical Center, Springfield, Massachusetts.
C N
CN
Otis
R A
RA
Fischer
N
N
Johnson
J F
JF
Kelleher
J L
JL
Powell
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