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Vulvodynia: A Common and Under-Recognized Pain Disorder in Women and Female Adolescents -- Integrating Current Knowledge into Clinical Practice

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These photos show vulvas with squamous cell carcinoma (SCC) on a background of LS. SCC can develop in 3-5% of women with LS. An additional cause of vulvar SCC is HPV infection, which can cause vulvar intraepithelial neoplasia and then progress to SCC. Additionally, melanoma and Paget disease can occur on the vulva. Therefore, in any patient with persistent ulceration, a mass or irregular pigmented lesion, the clinician should perform a vulvar biopsy to rule out malignancy. 

 

 

 

 

The MRI on the left shows a Tarlov cyst, a CSF-filled sac located in the spinal canal at the S1-S4 region of the spinal cord. Patients with Tarlov cysts are usually asymptomatic, but some do experience radiating pelvic and urogenital pain, persistent genital arousal disorder (PGAD), sexual dysfunction and bladder dysfunction, among other symptoms.

The image on the right shows a labral hip tear, which can refer pain to the urogenital region.

 

 

More than 130 million women across the world have undergone Female Genital Cutting (FGC) and in some countries prevalence of FGC is greater than 85% (UNICEF 2014).

Perineal trauma from a poorly healed or poorly repaired laceration or episiotomy may cause chronic vulvar pain or recurrent tearing (and pain) during intercourse. A recent review showed that 12.8% of women experience chronic vulvar pain at least five months after episiotomy (Turmo 2015).

Lastly, straddle injuries (most commonly from bicycles or falls from climbing) or injuries to the pelvis from motor vehicle accidents are uncommonly the cause of persistent vulvar pain.  

 

 

It has been recognized for decades that the tissues of the vulva and vagina are both responsive and dependent on sex steroids (hormones) for proper health and function. These sex steroids exert their effects through the nuclear receptors of estrogen, progesterone, androgen and glucocorticoid . The hormone receptors are very abundant in the epithelium of the outer vulva, endothelium of the vestibule, and mesothelium of the vagina. In addition, these hormone receptors are also found in high concentration in the deeper tissues of the vulva and vagina, including the glands and connective tissue, and adjacent to blood vessels and nerves. The hormones bind to the hormone receptors, which then leads to transcription and production of proteins that are essential for genital health and normal sexual function. Conversely, a deficiency in the sex steroids leads to dysfunction and frequently dyspareunia. It has been very well documented that a deficiency in circulating estrogen leads to anatomic and physiologic changes in the vagina. Anatomic changes include reduced collagen density and hyalinization, decreased elastin, thinning of the epithelium, altered appearance and function of smooth muscle cells, increased density of connective tissue, and fewer blood vessels. The labia minora thin and regress, the vestibule retracts, and the hymenal carunculae involute and lose elasticity. The urethral meatus appears prominent relative to the vestibule and becomes vulnerable to physical irritation and trauma. Physiologic changes result in reduced vaginal blood flow, diminished lubrication, decreased flexibility and elasticity of the vaginal vault, and increased vaginal pH. Furthermore, decreases in vaginal tissue strength and increased friability may predispose to epithelial damage with penetrative sexual activity, leading to pain, burning, tearing, irritation, and post-coital bleeding. Epithelial thinning with decreased glycogenated superficial cells leads to changes in vaginal flora and loss of lactobacilli, increased pH, and a change in the microbiome. More recently, it has become apparent that deficiency in circulating free testosterone also adversely affects the tissues of the vulva and vagina. Decreased levels of androgens are associated with decreased mucinification, decreased nerve fiber density, and decreased vasodilatation.

 

 

As seen in these photographs, women who receive adjuvant endocrine therapy after the diagnosis of breast cancer (with an aromatase inhibitor or Tamoxifen), have a significant increase in dyspareunia (Baumgart 2013).

Prophylactic oophorectomy in women with BRCA mutations also increases the rate of dyspareunia (Finch 2011).

Sixty-seven percent of women who receive pelvic radiation therapy for anogenital malignancies develop dyspareunia (Stinesen 2015).

Repair of pelvic organ prolapse with synthetic vaginal mesh may have significant complications, including mesh contraction; mesh erosion into the vagina, bladder, and bowel; and dyspareunia. A meta-analysis of 71 papers showed that the overall rate of chronic dyspareunia after mesh placement is 9.1% (Abed 2011).

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