Intraepidermal Paget disease may persist for years or even decades without evidence of invasion. However, when there is an associated tumor involving skin appendages, the Paget cells may invade locally and ultimately metastasize. After metastasis occurs, the prognosis is poor.
HPV-relatedvulvarsquamouscellcarcinomasusuallyarepoorlydifferentiatedlesionsandsometimesaremultifocal.Theyoftenevolvefromvulvarintraepithelialneoplasia.
Non–HPV-relatedvulvarsquamouscellcarcinomasoccurinolderwomenandusuallyarewelldifferentiatedandunifocal.Theyareoftenprecededby“differentiated”vulvarintraepithelialneoplasiaassociatedwithlichensclerosus.
VulvarPagetdiseaseischaracterizedbyared,scalyplaquecausedbyproliferationofepithelialcellswithintheepidermis;usually,thereisnounderlyingcarcinoma,unlikePagetdiseaseofthenipple.
In adult females, the vagina is seldom a site of primary disease. More often, it is involved secondarily by cancer or infections arising in adjacent organs (e.g., cervix, vulva, bladder, rectum).
Congenital anomalies of the vagina fortunately are uncommon and include entities such as total absence of the vagina, a septate or double vagina (usually associated with a septate cervix and, sometimes, septate uterus), and congenital, lateral Gartner duct cysts arising from persistent wolffian duct rests.
Vaginitis is a common condition that is usually transient and of no clinical consequence. It is associated with the production of a vaginal discharge (leukorrhea). A large variety of organisms have been implicated, including bacteria, fungi, and parasites. Many are normal commensals that become pathogenic only in the setting of diabetes, systemic antibiotic therapy (which causes disruption of normal microbial flora), immunodeficiency, pregnancy, or recent abortion. In adults, primary gonorrheal infection of the vagina is uncommon. The other organisms worthy of mention, because they are frequent offenders, are Candida albicans and Trichomonas vaginalis. Candidal (monilial) vaginitis is characterized by a curdy white discharge. This organism is part of the normal vaginal flora in about 5% of women, so the appearance of symptomatic infection almost always involves one of the predisposing influences listed above or superinfection by a new, more aggressive strain. T. vaginalis is sexually transmitted; it produces a watery, copious gray-green discharge in which parasites can be identified by microscopy. Trichomonas also can be identified in about 10% of asymptomatic women; thus, active infection usually stems from sexual transmission of a new strain.
Squamous cell carcinoma of the vagina is an extremely uncommon cancer that usually occurs in women older than 60 years in the setting of risk factors similar to those associated with carcinoma of the cervix (discussed later). Vaginal intraepithelial neoplasia (VAIN) is a precursor lesion that is nearly always associated with HPV infection. Invasive squamous cell carcinoma of the vagina is associated with the presence of HPV DNA in more than half of the cases, presumably derived from HPV-positive VAIN.