- Vulvar vestibulitis syndrome
- Primary neuroproliferative vestibulodynia / Congenital neuroproliferative vestibulodynia
- Secondary neuroproliferative vestibulodynia / Acquired neuroproliferative vestibulodynia
Vestibulodynia is a general term used to describe pain in the vestibule. The vestibule is the tissue within the vulva that sits at the opening of the vagina. The vestibule is the transition between external and internal, just like a vestibule of a building is the entrance.
Vestibulodynia can be considered a subcategory of vulvodynia. Vulvodynia is an even broader term used to describe chronic pain in the vulva. The terms vulvodynia and vestibulodynia are really only a description of symptoms, though they are often given as a diagnosis. Vulvodynia is typically used to describe generalized pain in the vulva, where the patient has chronic vulvar pain in external regions of the vulva rather than just in the vestibule. Ongoing research has improved our understanding of the underlying biological causes of pain, so terminology has changed and gotten more specific. ISSWSH, alongside two other international societies, published updated terminology in 2015.(1) We now recognize a few subtypes of vestibulodynia based on the root cause of the pain:
- Neuroproliferative vestibulodynia
- Hormonally-mediated vestibulodynia
- Inflammatory vestibulodynia
The vestibule tissue is fundamentally different from the skin around it, and even develops from a different part of the embryo. This inherent biological difference is crucial to understanding what causes pain specifically in the vestibule. In people with neuroproliferative vestibulodynia, there are too many nerve endings in the vestibule tissue because the nerves have proliferated, or replicated. Extra nerve endings have been visualized by microscopy of vestibule tissue samples, and researchers saw ten times more nerve endings than normal.(2) The cause of the neuroproliferation is still under investigation (see below).
There are two kinds of neuroproliferative vestibulodynia: primary and secondary. Primary is another word for congenital. For people with primary neuroproliferative vestibulodynia, they have had pain their entire lives. For people with secondary neuroproliferative vestibulodynia, also called acquired neuroproliferative vestibulodynia, pain developed later in life, and they can remember being pain free. It can be helpful to distinguish between congenital and acquired neuroproliferative vestibulodynia when choosing which treatment options will likely be best (see below).
Signs & Symptoms
People with vestibulodynia feel pain at the entrance of the vagina, called the vestibule. There are many ways to describe the pain in the vestibule. People with neuroproliferative vestibulodynia may describe their pain as sharp, stinging, burning, and/or hypersensitivity. Pain can also be classified by when it occurs. Provoked vestibulodynia is vestibule pain that occurs with touch or pressure, while unprovoked pain occurs spontaneously. Sometimes it can be difficult to distinguish provoked from unprovoked pain before all the triggers are recognized, especially when they are seemingly simple things like sitting or wearing tight clothing.
Patients with vestibulodynia often feel pain any time that there is contact or pressure on the vestibule. They almost always feel pain with vaginal penetration (called dyspareunia), including pain with penetration, using a tampon, and speculum exams. Day-to-day activities that put pressure on the vestibule can also cause pain, including wearing tight clothing, sitting for long periods or on hard surfaces, and wiping with toilet paper. A sign of congenital neuroproliferative vestibulodynia, and not secondary, is pain in the belly button, called umbilical hypersensitivity. About 60% of patients with primary neuroproliferative vestibulodynia have belly button pain.(3) Some patients describe their belly button pain as “zapping” down into their vulva. This is thought to happen because the belly button is made up of the same embryologic tissue as the vestibule, called endodermal tissue.
Many people with vestibulodynia will also have tight muscles in the pelvic floor, referred to as hypertonic pelvic floor dysfunction. In people with hypertonic pelvic floor dysfunction, the muscles in the pelvic floor are tight, which can cause pain and spasming. In some scenarios, spasms of the pelvic floor muscles is called vaginismus. We think pelvic floor dysfunction may develop in people with vestibulodynia due to a subconscious guarding response against pain. When you are in pain or anticipating pain, your muscles tense up to protect you. In many cases the response in helpful; for example, what would happen if you put your hand on a hot stove? Your muscles would contract without you telling them to. However, chronic tightening of the pelvic floor muscles only creates more pain. The muscles can accumulate knots (trigger points) and become shortened and weak. Additional symptoms of pelvic floor dysfunction can include feeling tension, pain, and burning in the hips, legs, lower back, and vulva, especially in the vestibule. You can also experience urinary symptoms like frequency, urgency, and leakage, as well as bowel symptoms like constipation or pain with bowel movements. Hypertonic pelvic floor dysfunction alone can be a cause of vestibulodynia, and it is important to determine the root cause of the vestibulodynia.
The vestibule tissue is fundamentally different from the skin around it, and even develops from a different part of the embryo. This inherent biological difference is crucial to understanding what causes pain specifically in the vestibule. The vestibule is embryonically derived from the primitive urogenital sinus and is endodermal tissue. In contrast, the external labia minora, like all skin, is ectodermal tissue. The delineation between the two is called Hart’s line.
In people with primary neuroproliferative vestibulodynia, where pain has been present forever, it is believed that there is a congenital birth defect and the excess nerve endings in the vestibule developed very early. This theory fits with the symptom of belly button (umbilical) hypersensitivity that is seen in many patients with primary neuroproliferative vestibulodynia. Both the vestibule and belly button develop from the same embryonic tissue (primitive urogenital sinus), so this symptom is evidence that there is an inherent problem with this tissue.(3)
Secondary neuroproliferative vestibulodynia develops later in life, and research is ongoing to further our understanding of how this happens. It is thought that the overgrowth of nerves is driven by the immune response, either to infection or allergy. In addition to finding increased numbers of nerve cells in the painful vestibule of patients with vestibulodynia, researchers also see increased numbers of immune cells.(2) Recent findings suggests that the nerve proliferation is triggered by signaling from immune cells in the tissue.(4) More work is needed to understand the details of this mechanism and why is occurs in some people but not others.
Anyone with a vestibule can have vestibulodynia. It is difficult to estimate the prevalence of vestibulodynia, but studies using questionnaires of the general population in the US found that 8-10% of women have chronic vulvar or vestibule pain causing pain with intercourse. (5, 6) In these two studies 20-40% of respondents reported pain with their first attempt to use a tampon, which may suggest that congenital neuroproliferative vestibulodynia makes up a significant proportion of this population. Hormonally-mediated vestibulodynia is similar to the condition experienced by people in menopause, termed genitourinary syndrome of menopause (GSM), so older women are more likely to experience vestibulodynia in that way. Unfortunately, it often takes a long time and multiple providers for patients to be accurately diagnosed with vestibulodynia, so patients are usually adults when diagnosed.(5)
First, a knowledgeable provider should do a thorough history of your story. A patient’s story holds important clues to their diagnosis. People with congenital neuroproliferative vestibulodynia will have pain when they first attempt to use tampons, pelvic exams, and penetrative sex. They are more likely to have learned to do day-to-day activities in ways that reduce their pain without ever realizing it; for example, patting rather than wiping with toilet paper or sitting in ways that reduce pressure on the vulva. Patients with acquired neuroproliferative vestibulodynia often have a history of chronic yeast infections or severe or repeated allergic reactions. Patients with hormonally-mediated vestibulodynia develop pain after starting a medication that affects hormone levels (hormonal birth control pills, spironolactone, tamoxifen), surgery (hysterectomy, oophorectomy), or while breastfeeding.
Then there should be a very specific exam of the pelvis, vulva, and vagina. In patients with neuroproliferative vestibulodynia, the vestibule usually appears healthy, without redness or signs of inflammation or irritation. The provider should manually examine the pelvic floor muscles to determine if there is excess tension in the muscles. The provider should feel the muscles externally and internally through the vagina. Many patients with vestibulodynia have tension in their pelvic floor, called hypertonic pelvic floor dysfunction. Tight muscles in the pelvic floor can be the sole cause of vestibulodynia or in combination with another cause of vestibulodynia.
There are a few tests important for diagnosing vestibulodynia. A process called a Q-tip test is important for mapping pain in the vulva. The provider will gently press a cotton swab to each part of the vulva while the patient reports their degree of pain at each spot. It is important that the provider touch each region of the vestibule, all the way around the vaginal opening. Locations in the vestibule are referred to using the numbers like a clock, with 12 o’clock at the top above the urethra and 6 o’clock at the bottom closest to the perineum. Patients with neuroproliferative vestibulodynia typically have pain throughout the vestibule, while patients with only hypertonic pelvic floor dysfunction will have pain in the lower half of the vestibule that is worst at 6 o’clock. Patients with hormonally-mediated vestibulodynia typically have pain throughout the vestibule as well, but with redness and signs of tissue atrophy.
Another test that can be helpful when diagnosing vestibulodynia is called a vestibular anesthesia test (VAT).(7) The purpose of the VAT is to test if numbing the vestibule tissue makes the pain go away. Vulvar pain can also be caused by nerve pinching or damage further up in the nerve. Because sensation in the vulva comes through the pudendal nerve, vulvar pain caused by problems in the nerve is called pudendal neuralgia. In patients with nerve problems, numbing the nerve endings during the VAT does not relieve pain, and only a pudendal nerve block that acts above the damaged site to turn off all sensation through the nerve can relieve pain. It is not common for pudendal nerve issues to cause pain that is only in the vestibule. During a VAT, local anesthetic is applied topically in a cream or by injection. Once the numbing is in full effect, then the Q-tip test is redone, as well as other things that would normally cause pain, like touch or penetration. If the pain is relieved, then the test is considered positive and indicates that the pain is coming from the vestibule tissue itself.
Data to help patients and their providers choose the best treatments for vestibulodynia is growing. Because the subtypes of vestibulodynia that are separated by the biological cause of pain were officially recognized relatively recently, most studies include patients with various kinds of vestibulodynia. While we wait for randomized placebo-controlled studies with distinct patient populations, we rely on the data that we do have, as well as the vast experience of specialists in sexual medicine who have successfully treated thousands of patients.
Many patients with vestibulodynia are offered topical lidocaine. Lidocaine gel can be pre-mixed or made by a compounding pharmacy. Custom compounded medications are especially helpful if someone is sensitive to a medication’s base, the inactive ingredients, and needs a different base. Controlled studies haven’t shown significant benefits for topical lidocaine treatment.(8) Topical lidocaine can be a helpful tool for enduring specific activities but only relieves pain temporarily and doesn’t fix the underlying problem. Another topical medication that targets the nerves is gabapentin. However, the clinical trial data and anecdotal evidence for its effectiveness is weak.(9) Additionally, the mechanism of action of the drug on the peripheral nerves is not well understood.
Capsaicin cream is a topical treatment that takes a slightly different approach.(8) Capsaicin is the chemical compound in peppers that makes them spicy. Patients apply capsaicin cream to the vestibule daily for longer and longer amounts of time, starting with only a few seconds by washing it off immediately. Over time, patients build up the amount of time they can tolerate the cream. The concept behind this treatment is that the capsaicin overloads the nerve endings and reduces their function. After 12 weeks, the intense hypersensitivity from having excess nerves in the vestibule is weakened.(10) We don’t have good data for the long-term efficacy of capsaicin, and some patients do need to keep using it to maintain the effect. We also need data comparing the efficacy of capsaicin treatment in patients with primary versus secondary neuroproliferative vestibulodynia. An obvious barrier to using this treatment is that it causes more pain, even if you use lidocaine before applying the capsaicin. For some patients, this might not be a good option for their mental health, and some providers do not routinely offer it.
Surgery is also a treatment option for neuroproliferative vestibulodynia. A vestibulectomy is a surgery to remove the vestibule tissue.(11, 12) The top experts believe that the entire vestibule should be removed and do not do partial vestibulectomy. If the patient truly has neuroproliferative vestibulodynia, then based on our understanding of the biological cause, then neuroproliferation and pain occurs throughout the entire vestibule. Vestibulectomy has a very high rate of success in patients with congenital neuroproliferative vestibulodynia and is considered the first-choice treatment by some top experts.(13, 14) Vestibulectomy is also an option for patients with acquired neuroproliferative vestibulodynia, but usually after simpler treatments fail.
For nearly all people with vestibulodynia, referrals for pelvic floor physical therapy and cognitive behavioral therapy are important aspects of treatment. Most people with vestibulodynia have some degree of pelvic floor dysfunction and pelvic physical therapy is the best way to assess and improve the pelvic floor. All sexual dysfunctions, and especially sexual pain, can cause significant psychological distress to patients.(15) It is important that treatment has a biopsychosocial approach that considers all aspects of the patient as a complete person. Cognitive behavioral therapy with a therapist with specialized training in sexual health is also important for healing and navigating the impacts of sexual dysfunction on quality of life and relationships. A multimodal approach is most likely to be successful, so patient should engage with a team of providers throughout their journey.
There are many societies with interests in female sexual dysfunctions in addition to ISSWSH. The National Vulvodynia Association (NVA) is a US-based association founded by patient advocates that focuses specifically on vulvodynia. They provide some educational materials for the public and fund research of vulvodynia. Female sexual dysfunctions fall under the umbrella of interests of additional societies as well: International Society for the Study of Vulvovaginal Diseases (ISSVD), Sexual Medicine Society of North America (SMSNA), International Pelvic Pain Society (IPPS), International Society for Sexual Medicine (ISSM), and American Association of Sexuality Educators, Counselors and Therapists (AASECT), for example.
There are many online support groups and communities of patients who support each other on social media. To find fellow patients locally, ask a provider if they know of another patient seeking community or any local groups to join.
- Bornstein J, Goldstein AT, Stockdale CK, Bergeron S, Pukall C, Zolnoun D, Coady D, consensus vulvar pain terminology committee of the International Society for the Study of Vulvovaginal D, International Society for the Study of Women's Sexual H, International Pelvic Pain S. 2016. 2015 ISSVD, ISSWSH, and IPPS Consensus Terminology and Classification of Persistent Vulvar Pain and Vulvodynia. J Sex Med 13:607-12.
- Bornstein J, Goldschmid N, Sabo E. 2004. Hyperinnervation and mast cell activation may be used as histopathologic diagnostic criteria for vulvar vestibulitis. Gynecol Obstet Invest 58:171-8.
- Burrows LJ, Klingman D, Pukall CF, Goldstein AT. 2008. Umbilical hypersensitivity in women with primary vestibulodynia. J Reprod Med 53:413-6.
- Barry CM, Matusica D, Haberberger RV. 2019. Emerging Evidence of Macrophage Contribution to Hyperinnervation and Nociceptor Sensitization in Vulvodynia. Front Mol Neurosci 12:186.
- Harlow BL, Kunitz CG, Nguyen RH, Rydell SA, Turner RM, MacLehose RF. 2014. Prevalence of symptoms consistent with a diagnosis of vulvodynia: population-based estimates from 2 geographic regions. Am J Obstet Gynecol 210:40 e1-8.
- Reed BD, Harlow SD, Sen A, Legocki LJ, Edwards RM, Arato N, Haefner HK. 2012. Prevalence and demographic characteristics of vulvodynia in a population-based sample. Am J Obstet Gynecol 206:170 e1-9.
- Gagnon C, Minton J, Goldstein I. 2014. Vestibular anesthesia test for neuroproliferative vestibulodynia. J Sex Med 11:1888-91.
- Rosen NO, Dawson SJ, Brooks M, Kellogg-Spadt S. 2019. Treatment of Vulvodynia: Pharmacological and Non-Pharmacological Approaches. Drugs 79:483-493.
- Brown CS, Bachmann GA, Wan J, Foster DC, Gabapentin Study G. 2018. Gabapentin for the Treatment of Vulvodynia: A Randomized Controlled Trial. Obstet Gynecol 131:1000-1007.
- Steinberg AC, Oyama IA, Rejba AE, Kellogg-Spadt S, Whitmore KE. 2005. Capsaicin for the treatment of vulvar vestibulitis. Am J Obstet Gynecol 192:1549-53.
- Wu C, Goldstein A, Klebanoff JS, Moawad GN. 2019. Surgical management of neuroproliferative-associated vestibulodynia: a tutorial on vestibulectomy with vaginal advancement flap. Am J Obstet Gynecol 221:525 e1-525 e2.
- Michelle A. King RR, Andrew T. Goldstein. 2014. Current uses of surgery in the treatment of genital pain. Current Sexual Health Reports 6:252-258.
- Irwin Goldstein (Editor) AHCE, Andrew T. Goldstein (Editor), Noel N. Kim (Editor), Sheryl A. Kingsberg (Editor). 2018. Textbook of Female Sexual Function and Dysfunction: Diagnosis and Treatment, 1 ed. Wiley-Blackwell.
- Michelle A. King LSM, Zoe Belkin, Andrew T. Goldstein. 2018. Vulvar Vestibulectomy for Neuroproliferative-Associated Provoked Vestibulodynia: A Retrospective Analysis. Journal of Gynecologic Surgery 34.
- Chisari C, Monajemi MB, Scott W, Moss-Morris R, McCracken LM. 2021. Psychosocial factors associated with pain and sexual function in women with Vulvodynia: A systematic review. Eur J Pain 25:39-50.