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Vulvar and Vaginal Pathologies

Vulvar and Vaginal Pathologies
Here we'll learn about pathologies of the vulva and vagina; please note that congenital anomalies and sexually transmitted infections are covered in detail, elsewhere.
Vaginal orifice (opening)
Remnants of the hymen with a fimbriated appearance, but it can take many forms, including annular and crescentic. The hymen is a non-functional embryological remnant that partially covers the vagina at birth, but stretches over time from physical activity, tampon use, etc.
Although it's commonly stated that the hymen closes off the vagina until "broken" by sexual penetration, this is untrue; the hymen opens long before sexual activity, and is not an indicator of someone's sexual history.
Ducts of the greater vestibular glands, aka, Bartholin glands, which release lubricating mucous secretions.
The urethral opening
Paraurethral glands (aka, lesser vestibular glands, aka, Skene's glands); these also release lubricating mucous secretions.
Glans of the clitoris, which is the "free" portion of erectile tissue; the majority of the clitoris is deep to the skin.
Labia minora; medial and lateral folds of the labia minora; they comprise erectile tissue and rich vasculature covered by glycogen-rich stratified epithelia. The lateral folds meet anteriorly to form the prepuce (aka, hood), which covers of the body of the clitoris.
The area between the labia minora is the vestibule of the vagina (hence, the greater and lesser "vestibular" glands we drew earlier).
Labia majora comprise adipose and smooth muscle tissue covered by keratinized skin; they are embryologically analogous to the skin of the scrotum, with hair follicles and sebaceous glands.
The labia majora meet anteriorly at the mons pubis, which is the mound of fatty tissue superficial to the pubic symphysis.
Vulvar Pathologies, Non-Neoplastic
Bartholin cysts
These cysts form when a Bartholin gland (greater vestibular glands) becomes blocked, and mucus accumulates under the tissue. These cysts are usually asymptomatic, but can be irritating if large in size.
When Bartholin cysts become infected, they become abscesses, which can cause vulvar pain and redness, and occasionally, fever.
Bartholin cysts in patients under 40 years old do not require specific treatment, and sitz baths are recommended for irritation.
Abscesses can be drained, and further surgical interventions may be needed to prevent recurrence; antibiotics can also be prescribed.
In patients older than 40 years old, new cysts should be biopsied or removed to rule out vulvar cancer. Lichen sclerosus
This is a chronic, progressive skin condition that presents with thinning skin and porcelain white plaques that may bleed. We show the classic sign of a figure-eight pattern around the genitals and perianal region.
Patients experience itching and pain upon intercourse, urination, and defecation.
Lichen sclerosus is thought to be an autoimmune condition with genetic underpinnings.
It is a risk factor for vulvar squamous cell carcinoma.
Lichen simplex chronicus
Aaka, chronic neurodermatitis
This is characterized by thick, scaly, itchy plaques that form in response to chronic irritation related to rubbing or scratching the area (patches also appear in other areas of the body, most commonly the back of the neck, forearms, legs).
Although heat, sweat, and friction can be the primary source of irritation that leads to chronic scratching, we also see lichen simplex chronicus in patients who scratch or rub their skin in response to psychological distress (anxiety, obsessive compulsive disorder).
Vulvar Pathologies, Neoplastic
These are rare, and more common in postmenopausal women.
Vulvar Carcinoma
Patients most often present with itchy, painful vulvar lumps that typically originate in the labia majora.
Most vulvar carcinomas are squamous cell; the precursor lesion is a high grade squamous intraepithelial lesion.
A major risk factor for developing high grade squamous intraepithelial lesions and squamous cell vulvar carcinoma is persistent infection by HPV, subtype 16.
Be aware that HGSIL was formerly called VIN, vulvar intraepithelial neoplasia.
When diagnosed in earlier stages, we can treat vulvar carcinoma with surgical excision and lymph node dissection; in later stages, surgery is followed by chemotherapy and radiation.
Extramammary Paget disease
This manifests as intraepithelial adenocarcinoma, often on the labia majora (note that lesions also appear in non-genital areas of the body).
Patients have itchy, diffuse red plaques that may weep and crust; surgical excision is the primary treatment option.
Vaginal Pathologies, Non-Neoplastic
Imperforate hymen
This is a rare condition in which the hymen completely covers the vaginal opening.
In these patients, uterovaginal secretions and menstrual blood can accumulate in the vagina and cause pain.
Surgical incision of the imperforate hymen is corrective.
Vaginitis
Infection and inflammation of the vagina and vulva. Vaginitis is common, and one-third of women will experience some form of it in their lifetimes.
Bacterial vaginosis is the number one cause of vaginitis; it is caused by altered vaginal bacterial flora.
Signs and symptoms include vaginal pH greater than 4.5, a thin, watery, nonpurulent discharge, and a positive amine odor test.
On wet mount, we'll see Clue cells, which are vaginal epithelial cells covered with bacteria.
These infections can be treated with metronidazole.
Candida infection is the second most common cause of vaginitis.
This is what we commonly refer to as a "yeast infection," and is usually caused by Candida albicans.
Patients experience vulvar and vaginal itching and soreness, and have a thick, white vaginal discharge.
We can treat uncomplicated cases with topical antifungal agents or oral fluconazole.
Most patients are asymptomatic, but they may present with a yellowish green malodorous vaginal discharge and vulvar irritation.
The vaginal mucosa and cervix may also be involved; on physical exam, we look for "strawberry cervix," which is characterized by erythema with spots of hemorrhagic spotting.
Vaginal Pathologies, Neoplastic
Primary vaginal cancer is rare; most vaginal carcinomas are metastases from other sites.
The most common subtype of primary vaginal cancer is squamous cell carcinoma.
Usually diagnosed in post-menopausal patients who present with vaginal bleeding, malodorous discharge, and pain.
Vaginal tumors can extend directly into the surrounding soft-tissue structures, and, in later stages, spread hematogenously to the liver, lungs, and other organs.
As in vulvar cancer, persistent infection by HPV subtype 16 is a major risk factor for squamous cell carcinoma of the vagina and its precursor lesion.
Recall that the HPV vaccine, which also prevents cervical cancer, protects against HPV subtype 16.
For references, please see full references.