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Vulvar & Vaginal Lesions

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Pathophysiology

Summary

The Bartholin glands are secretory glands located in the vulva that produce a mucoid secretion that aids in lubrication. A blockage in a Bartholin duct can cause the gland to dilate with fluid, leading to a Bartholin cyst. When infected, this cysts evolve into a Bartholin abscess.

Various dermatoses collectively called 'vulvar lichens' can afflict the vulvar region, each of which present with pruritus, and noticeable skin changes. Lichen sclerosus manifests as smooth white plaques on thin, ‘parchment-like’ tissue, and carries a small risk of progression to vulvar squamous cell carcinoma. Histology shows thinned epidermis overlying sclerotic stroma. Lichen planus is a dermatosis that occurs in the vulvar area as well as the oral mucosa & non-vulvar skin. It appears as fine, net-like white plaques known as Wickham striae in the mouth, and as purple polygonal papules on the skin. Lichen simplex chronicus is primarily characterized by severe pruritus leading to chronic scratching, and presents as leathery white plaques with excoriations. Histology shows epithelial thickening due to squamous cell hyperplasia and hyperkeratosis.

Vulvar intraepithelial neoplasia (VIN) serves as a precursor to vulvar squamous cell carcinoma. Analogous to cervical intraepithelial neoplasia (CIN), VIN is caused by high-risk HPV subtypes like 16 & 18. It is categorized into VIN 1, 2, and 3, based on the depth of abnormal cells and degree of dysplasia. Progression to vulvar squamous cell carcinoma is a serious risk, identifiable by keratin pearls and intercellular bridges on histology.

Rare vaginal cancers include embryonal rhabdomyosarcoma is a serious form of skeletal muscle cancer that presents as a jelly-like cystic mass protruding from the vagina, usually in young girls. Clear cell adenocarcinoma is characterized histologically by cells with clear, vacuolated cytoplasm. In contrast, vaginal adenosis features columnar epithelium in the exocervix and vagina instead of the typical squamous epithelium. Women exposed to diethylstilbestrol (DES), a synthetic form of estrogen, in utero have an increased risk of clear cell adenocarcinoma and vaginal adenosis, as well as other uterine abnormalities.

Lastly, Gartner duct cysts, which are Wolffian duct remnants, may appear on the lateral vaginal wall

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FAQs

What are Bartholin glands and what happens when a Bartholin duct is blocked?

Bartholin glands are secretory glands located in the vulva that produce a mucoid secretion that aids in vaginal and vulvar lubrication. When a Bartholin duct becomes blocked, the gland may fill with fluid, leading to the formation of a Bartholin cyst. If this cyst becomes infected, it can develop into a Bartholin abscess, which may require medical intervention.

How do vulvar lichen conditions manifest and what are their distinguishing histological characteristics?

Lichen sclerosus, lichen planus, and lichen simplex chronicus are benign skin conditions that affect the vulva. All three conditions present with severe itching (pruritus) and distinct visual skin changes. Lichen sclerosus manifests as smooth white plaques on thin, ‘parchment-like’ tissue and is histologically characterized by a thinned epidermis overlying sclerotic stroma. Lichen planus can appear as fine net-like white plaques, known as Wickham striae, in the oral mucosa or as purple polygonal papules on the skin. Lichen simplex chronicus results from chronic scratching and appears as leathery white plaques with excoriations; its histology shows epithelial thickening due to squamous cell hyperplasia and hyperkeratosis.

What is vulvar intraepithelial neoplasia (VIN) and how is it graded?

Vulvar intraepithelial neoplasia (VIN) is a precancerous condition analogous to cervical intraepithelial neoplasia (CIN). It is primarily caused by high-risk HPV subtypes, such as 16 and 18. VIN is categorized into three grades: VIN 1, VIN 2, and VIN 3, based on the depth of abnormal cells and the degree of dysplasia present. VIN has the potential to progress to vulvar squamous cell carcinoma, which histologically features keratin pearls and intercellular bridges.

What is embryonal rhabdomyosarcoma and how does it present?

Embryonal rhabdomyosarcoma is a rare form of skeletal muscle cancer that primarily affects young girls. It commonly presents as a jelly-like cystic mass protruding from the vagina.

What is the relationship between diethylstilbestrol (DES) exposure and the risk of clear cell adenocarcinoma and vaginal adenosis?

Diethylstilbestrol (DES) is a synthetic estrogen that, when administered to pregnant women, increases the risk of certain conditions in their daughters. Women exposed to DES in utero have an elevated risk for developing clear cell adenocarcinoma, which is characterized histologically by clear vacuolated cells. They are also more likely to develop vaginal adenosis, a condition where the exocervix and vagina have columnar rather than squamous epithelium. Additionally, in utero DES exposure can lead to uterine and cervical anomalies.