Version May 2024
INTRODUCTION — Localized vulvar pain syndrome is a persistent vulvar pain that can be consistently and precisely localized to the vulvar vestibule during physical examination, has no identifiable cause, and has been present for at least three months. This syndrome has previously been referred to as vulvodynia, vestibulodynia, vulvar vestibulitis, and focal vulvitis.
This topic will review the clinical manifestations and diagnosis of localized vulvar pain syndrome. Information on treatment of vulvar pain, generalized vulvar pain syndromes, and sexual pain in women is presented separately.
●(See "Female sexual pain: Evaluation".)
●(See "Female sexual pain: Differential diagnosis".)
TERMINOLOGY — In 2015, representatives from the International Society for the Study of Vulvovaginal Disease, the International Society for the Study of Women's Sexual Health, and the International Pelvic Pain Society developed consensus terminology and classified persistent vulvar pain into two categories [1]:
●Vulvar pain caused by a specific disorder
●Vulvodynia (ie, persistent vulvar pain without an identifiable etiology)
Examples of vulvar pain caused by a specific disorder include infectious, inflammatory, neoplastic, and neurologic processes (table 1). By contrast, vulvodynia is defined as vulvar pain of at least three months duration that has no identifiable cause.
Vulvodynia can be further subdivided by [2]:
●Location – The symptoms can be localized (eg, vestibulodynia, clitorodynia), generalized, or mixed (localized and generalized)
●Provocation – Provoked (eg, insertional, contact), spontaneous, or mixed (provoked and spontaneous)
●Onset – Primary or secondary (primary symptoms have always been present, such as with tampon insertion or sexual intercourse; secondary symptoms develop after a period of normal function)
●Temporal pattern – Intermittent, persistent, constant, immediate (ie, symptomatic immediately after physical contact), or delayed (symptoms delayed after contact)
Localized vulvar pain syndrome is the most common vulvodynia and may include the clitoris (clitorodynia) as well as the vulvar vestibule (vestibulodynia) (see 'Diagnostic criteria' below). Localized vulvar pain syndrome is typically provoked or worsened with direct touch, but may occur spontaneously.
EPIDEMIOLOGY — Although localized vulvar pain syndrome appears to be the most common subtype of chronic vulvar pain of unknown etiology [3], the incidence and prevalence are not known because of the factors below and because the diagnostic criteria have changed (erythema is no longer included in the diagnosis [4,5]). The prevalence of chronic vulvar pain (all types) ranges from 3 to 15 percent in self-report studies [6-9]. Assessing prevalence is challenging because many women with vulvar pain do not present for evaluation and the condition is frequently misdiagnosed when they are seen [10]. Additionally, patients presenting with vestibular pain may have heterogenous presentations with other chronic overlapping pain conditions, leading to generalized pain at other sites within the pelvis and exacerbated by contributing factors such as vulvovaginal infections, hypoestrogenic states, and disorders related to anxiety, depression, catastrophizing, and other psychosocial factors [11,12].
Vulvodynia is considered the most frequent cause of dyspareunia among premenopausal women, with an estimated prevalence of 12 percent among community-dwelling women [13]. In a study of over 2250 women who completed a self-administered validated questionnaire, just under one-half of the women meeting criteria for vulvodynia sought treatment, and only 1.4 percent of the women evaluated were actually diagnosed with vulvodynia [14]. A different population-based study of 441 women who screened positive for vulvodynia reported that 51 percent experienced remission over 6 to 30 months (with no relapse) while 10 percent had persistent symptoms [15]. The need for careful inclusion criteria when studying vulvodynia is highlighted by a retrospective case review of 525 women with isolated vulvar pain in which over 60 percent were diagnosed with a dermatologic condition that responded to appropriate treatment; these patients do not meet the criteria for vulvar pain of unknown cause [16]. (See 'Terminology' above.)
PATHOGENESIS AND LIKELY CONTRIBUTORS — The etiology of localized vulvar pain syndrome is not fully known and is likely multifactorial [10,17]. The leading hypothesis is that an inciting event, such as infection or trauma, initiates a local inflammatory response [3]. In susceptible women, the inflammatory response causes the proliferation of nerve fibers in the vestibular tissue and lowered pain thresholds, which can then lead to central sensitization of pain [18-21]. (See "Evaluation of chronic non-cancer pain in adults".)
Inflammation — An excessive or abnormal immune response has been postulated as a cause of vulvodynia, although the data are mixed. A systematic review of 18 studies, including 400 women, found limited and contradictory evidence for an association of local and systemic inflammation on localized vulvodynia [22]. Increased production of pro-inflammatory chemicals and/or limited ability of tissues to clear the inflammatory response could sensitize neurons in the vulvar vestibule and cause up-regulation of neurons in the central nervous system [10]. Studies have reported an increase in the inflammatory markers interleukin-6, interleukin-1, and tumor necrosis factor-alpha and a decrease in the macrophage inhibitory protein 1-beta [23-25]. The surface receptor Dectin-1, which binds Candida albicans, is also increased in women with vestibular pain compared with control women [26]. Therefore, women with vestibular pain syndromes may have an increased propensity to bind C. albicans and release an inflammatory chemical response compared with asymptomatic women.
While the above studies support the role of an abnormal immune response in women with localized vulvar pain syndrome, older studies have not noted differences among affected and asymptomatic women. Analyses of tissue biopsies reported similar nonspecific inflammatory cellular infiltrates, immunohistochemical results, and inflammatory mediators among women with localized vulvar pain syndrome and asymptomatic women [27-29]. However, these studies were limited by use of cellular and histochemical techniques, which are less sensitive than the molecular and genomic analyses performed in later studies. (See "Tools for genetics and genomics: Cytogenetics and molecular genetics".)
Neurologic proliferation and sensitization — In women with localized vulvar pain syndrome, studies have reported increased density of intra-epithelial nerve fibers [30,31], increased vanilloid receptor VR1 (which is triggered by the inflammatory response) [32], and hyperesthesia, which results from sensitization of the peripheral and central nervous systems [33,34]. Additionally, reduced sensory and pressure-pain thresholds have been reported in patients with localized vulvar pain syndrome [35-38]. Chronic inflammation of the vulvar vestibule may lead to hyperinnervation and sensitization such that even light touch to the vulva is transformed into a significantly painful response (allodynia) (figure 1). A retrospective study of 39 patients with refractory chronic pelvic pain reported approximately two-thirds of patients demonstrated small fiber polyneuropathy, as identified by lower extremity punch biopsy [39]. Additionally, pain can persist after the original infection or insult has cleared.
Infection — Studies have reported an association between genital infection, particularly with C. albicans and bacterial vaginosis, and localized vulvar pain syndrome [40,41]. Approximately 70 percent of women with vulvodynia report a history of frequent or severe yeast infections [4,42-44]. Additionally, increasing number of pelvic infections appears to increase the risk of having localized vulvar pain syndrome [40]. While these data are limited by small sample sizes and use of self-report as evidence of prior infection [40,41], an abnormal immune response to genital infection has been demonstrated in women with vulvodynia [23-25]. Other nonimmune contributors to postinfection pain syndromes likely include agent-specific characteristics, host susceptibility, altered vaginal microbiota, altered chemical metabolites produced by the disturbed flora (vaginal metabolome), development of abnormal pelvic floor muscle response during or after infection, and early central sensitization of pain [10].
Genetic factors — Genetic polymorphisms may impair the immune system response to trauma or infection, decrease the ability to terminate the inflammatory response, or increase the susceptibility to pain after exposure to infectious agents [10,45]. Genetic polymorphisms for interleukin-1 beta, tumor necrosis factor-alpha, interleukin-1 receptor antagonist, mannose-binding lectin, and melanocortin-1 receptor have been associated with vulvar pain [45-49]. Women with localized vulvar pain syndrome appear to produce a more severe inflammatory reaction (eg, increased interleukin-1 beta) and have alterations in their ability to resolve inflammatory reactions (eg, impaired interleukin-1 receptor antagonist) [50,51]. Increased cytosine-adenine-guanine (CAG) repeats in genes coding for androgen receptors have been noted in adolescents with vulvar pain that develops in response to combined hormonal contraceptives compared with control patients; these are postulated to be due to decreased free testosterone [52,53].
Allergy — Allergic reaction has been associated with local vulvar pain syndrome. In one study of 220 women with localized vulvar pain syndrome, 20 percent had a vaginal allergy (defined as elevated immunoglobulin E levels) and nearly half were successfully treated with antihistamine medications [54]. In a histopathologic study of 12 women with localized vulvar pain syndrome and 12 asymptomatic women, 75 percent of women with localized vulvar pain syndrome had immunoglobin G positive plasma cells compared with none in control women [55]. Lastly, in a study that performed skin patch testing with candida, women with vulvodynia needed lower concentrations of C. albicans to elicit a response and affected women produced a greater response compared with control women [56].
Hormonal — The impact of reproductive hormones on development of vulvodynia is not clear. Studies have reported both decreased [57] and increased [58] levels of estrogen receptor alpha in women with localized vulvar pain syndrome compared with control women. Similarly, data on the impact of contraceptive hormone pills on vestibular pain are conflicting. In two population-based studies, no significant association was found between past or current oral contraceptive use and adult-onset vulvodynia [59,60]. However, other studies reported lower mechanical pain thresholds and increased risk of developing vulvar vestibular pain with oral contraceptive use [61-64]. Women with weaker androgen receptors may be more susceptible to vulvodynia during hormonal contraceptive use [52]. Hormonal alterations may explain why obese women (body mass index [BMI] ≥30 kg/m2) are less likely to have resolution of localized vulvar pain syndrome than normal-weight women (BMI 18.5 to 24.9 kg/m2) [65].
Myofascial — Pelvic floor muscle dysfunction can exacerbate localized vulvar pain symptoms [66]. Additionally, pelvic myofascial disorders or pelvic floor hypertonus can develop in patients with vulvodynia because contact with the vulvar vestibule causes pain, which triggers a reflex contraction and resultant increased tension in the levator ani and other pelvic floor muscles [67,68]. (See "Myofascial pelvic pain syndrome in females: Clinical manifestations and diagnosis".)
Psychological dysfunction — The relationship between psychological dysfunction and localized vulvar pain syndrome is debated. Women with localized vulvar pain syndrome exhibit lower pain thresholds, increased systemic pain perception, anxiety, depression, hypervigilance, and somatization compared with women without vulvodynia [57,61,69,70]. However, overall, there is no evidence for a primary psychological cause for localized vulvar pain syndrome. Consequences of the mental health stigma associated with vulvar pain can include dismissive experiences within the health care system, feelings of shame and guilt, low self-esteem, social isolation, and negative social narratives surrounding sexuality and femininity [71]. The chronic pain associated with this condition can lead to secondary psychological and mental health disorders, and patients with psychiatric disorders or a history of sexual assault [72] may be more susceptible to chronic pain. (See "Evaluation of chronic non-cancer pain in adults", section on 'Psychiatric comorbidity'.)
Examples of the conflicting nature of the data include:
●In one large United-Kingdom-based retrospective cohort study of 22,604 adult women exposed to domestic abuse, compared with 44,671 matched controls (unexposed women), history of abuse was associated with the development of chronic lower back pain, chronic headaches, and irritable bowel syndrome but not with the development of vulvodynia [73].
●In a Swedish population registry of over one million women aged 15 to 43, early adverse events at birth such as preterm birth, low birth weight, and low Apgar scores were associated with the development of provoked vestibulodynia later in life [74].
DIAGNOSTIC CRITERIA — The criteria for localized vulvar pain syndrome include [10]:
●Pain localized to the vulvar vestibule, with or without the clitoris
●Absence of identifiable cause
●Duration of at least three months
●Pain elicited with pressure-point testing
Additionally, the timing of symptom onset can be primary (from initial vestibular contact) or secondary (develop after a period of normal function). Symptoms can have multiple temporal patterns (ie, intermittent, persistent [nonrelapsing], or constant [continuous]) and variable timing after vestibular contact (ie, immediate or delayed). The most common form of the disorder is provoked by genital contact in an otherwise asymptomatic woman. Other variants include women with spontaneous pain or both provoked and spontaneous symptoms.
Vulvar pain of known etiology and localized vulvar pain syndrome can coexist; women can have a specific vulvar disorder such as lichen sclerosus, herpes, or candida and have concomitant vulvodynia. These women are typically identified when their vulvodynia symptoms persist after treatment of all identified causes of vulvar pain.
CLINICAL PRESENTATION — The hallmark symptom in women with localized vulvar pain syndrome is significant pain upon contact with the vulvar vestibule (picture 1). Common clinical complaints include pain with vaginal intercourse, tampon insertion, tight clothing, prolonged sitting, biking, or other sports. Pain onset can be immediate or delayed and discomfort can persist or resolve on its own. Most affected women are between 20 and 60 years of age [10], but symptoms can begin in childhood or adolescence [18,75]. Additionally, the pain is sufficiently severe to limit sexual function, cause psychological distress, impair relationships, and/or adversely affect routine activities [10,17,76]. (See 'Diagnostic criteria' above.)
DIAGNOSTIC EVALUATION — The diagnostic evaluation consists of a detailed history and physical examination that includes focal pressure-point testing of the vulvar vestibule. One purpose of the evaluation is to identify potential specific causes of the woman's pain, such as infection or genitourinary syndrome of menopause. Localized vulvar pain syndrome is a diagnosis of exclusion; women with an identifiable cause of symptoms have specified vulvar pain and do not meet the defining criteria for localized vulvar pain syndrome. However, the diagnostic process can be confusing as vulvar pain from a specific cause can coexist with localized vulvar pain syndrome [1]. (See 'Diagnostic criteria' above.)
History — The history includes a comprehensive review of systems, detailed gynecologic history, and vulvar pain history to characterize the patient's pain and identifies potential specific causes of vulvar pain (table 1). The International Pelvic Pain Society has developed a history and physical examination form for evaluation of women with chronic pelvic pain of any etiology. During the review of systems, we specifically ask about prior skin disorders (any location), allergies, and diet/nutrition, as these processes can contribute to specified vulvar pain. Excessive urinary oxalate excretion has been purported as a vulvar irritant in patients with vulvodynia but has not been implicated as a cause of localized vulvar pain syndrome [77]. Some patients with localized vulvar pain syndrome find acidic or sugary foods exacerbate vulvar pain. Eliminating certain foods and monitoring pain symptoms can help discern any relationship between diet and localized vulvar pain.
The gynecologic history can identify other potential causes of the woman's symptoms as well as risk factors for localized vulvar pain syndrome. We ask about prior genital infection, topical treatments, and hormone exposure. Women with localized vulvar pain syndrome often have a history of multiple genital infections. Topical treatments, such as antifungal cream, can cause or worsen dermatitis, which is a specific cause of vulvar pain. Hormone use, particularly with oral contraceptive pills, has been associated with localized vulvar pain syndrome in some studies. In addition, we take a detailed sexual history, assess for female sexual dysfunction (desire, arousal, orgasm, pain), and screen for intimate partner violence. We inquire about pelvic floor dysfunction (eg, urinary or fecal incontinence, prolapse), obstetric injury, and prior abdominal/pelvic surgery. Rarely, renal stones can cause pain in the ipsilateral vulva. Both psychological and anatomic trauma can exacerbate or cause vulvodynia as well as cause specified vulvar pain such as vaginismus. The diagnosis of localized vulvar pain syndrome is made if identifiable specific causes of pelvic and vulvar pain have been treated (eg, antifungals for candida and pelvic floor physiotherapy for vaginismus), related psychological issues have been addressed, and the vestibular pain persists. (See "Overview of sexual dysfunction in females: Epidemiology, risk factors, and evaluation" and "Intimate partner violence: Diagnosis and screening" and "The gynecologic history and pelvic examination", section on 'Urinary incontinence and pelvic organ prolapse'.)
For the portion of the history focused on the vulvar vestibular symptoms, we specifically inquire about the following:
Pain characteristics
●Descriptors of pain – Women with localized vulvar pain syndrome can consistently describe, localize, and rate the pain. Nearly 90 percent of women with localized vulvar pain syndrome describe their symptoms as a burning sensation localized to the vulvar vestibule [5]. Other descriptors include sharp, cutting, hot, knife-like, "like razor blades," and throbbing. The pain can be primary (onset with first introital touch, such as sexual activity or tampon use) or secondary (occurring after a period of normal function) [10].
Location – An anatomic diagram that allows the woman to identify the sites of pain is helpful. Localized vulvar pain syndrome is limited to the vestibule or clitoris. Women who cannot locate the sites of pain likely have vulvar pain from another disorder or generalized vulvar pain.
Intensity – Additionally, we ask our patients to use a numerical 10-point pain scale to rate their symptoms over time. This helps us understand the duration and severity of pain as well as the impact of pain on the woman's daily activities. (See "Evaluation of chronic non-cancer pain in adults", section on 'Pain severity and impact'.)
●Timeline of pain – A timeline of pain onset and duration, antecedent events (eg, genital infection, tampon use, intercourse), and treatment is established. Symptoms can be sudden onset or delayed and continuous or intermittent. Symptoms must be present for at least three months to be considered localized vulvar pain syndrome. Women who have symptoms for less than three months are evaluated for vulvar pain from a specific cause (table 1).
Associated symptoms
●Dyspareunia – We ask if the woman has introital dyspareunia. Introital dyspareunia is one of the defining symptoms of vestibulodynia [52]. Women who have general dyspareunia or pain with deep penetration are evaluated for other causes for their symptoms, such as vaginismus or endometriosis. (See "Female sexual pain: Differential diagnosis".)
●Tampon use – Women with localized vulvar pain syndrome typically cannot use tampons because of the severe pain with insertion. In a questionnaire study of 30 women with vulvar vestibular pain, nearly half had pain with tampon insertion [42]. If a woman is able to regularly use tampons, she is less likely to have localized vulvar pain syndrome and we evaluate for other causes of her symptoms.
●Myofascial pain – Myofascial pain syndromes appear to be a risk factor for localized vulvar pain syndrome, and women who have localized vulvar pain syndrome can develop secondary myofascial pain because of progressive tension within the pelvic floor muscles. To screen for myofascial pain, we ask women if they have deep burning pain, pelvic heaviness, or referred pain to other sites. (See 'Myofascial' above.)
●Menses – In general, menses does not worsen localized vulvar pain syndrome. Women whose symptoms are worse with menses are evaluated for hormonally dependent causes of pelvic pain, such as endometriosis or pelvic congestion syndrome.
•(See "Vulvovaginal varicosities and pelvic congestion syndrome".)
•(See "Endometriosis: Clinical features, evaluation, and diagnosis", section on 'Clinical features'.)
●Symptoms with semen exposure – Women with localized vulvar pain and seminal plasma allergy can have similar presentations. The difference is women with localized vulvar pain syndrome have symptoms even with condom use but women with seminal plasma allergy do not. (See "Allergic reactions to seminal plasma".)
Psychosocial impact
●Psychosocial impact – Women with localized vulvar pain syndrome commonly have compromised sexual function, reduced psychological well-being, and resultant impaired interpersonal relationships. Additionally, routine activities can be affected. We ask women if they avoid or no longer perform certain activities because of their symptoms and how their symptoms impact their relationships. The International Pelvic Pain Society has the most comprehensive questionnaire that includes the McGill pain inventory, a picture of the vulva and vestibule for patients to mark painful areas.
Physical examination — The cardinal sign of localized vulvar pain syndrome is significant tenderness upon point-pressure testing of the vulvar vestibule. The physical examination also includes visual inspection of the genitalia, palpation of the introitus and vagina, and speculum examination of the vagina and cervix. Even in women with abnormal point-pressure testing of the vestibule, we complete the physical examination in order to exclude other possible causes of the woman's symptoms.
Inspection — A main goal of visual inspection is to identify other possible causes for the woman's symptoms, particularly infection, allergy, neoplasm, or dermatosis. We begin by looking at the mons, groin creases, labia majora and minora, clitoris (glans, hood, prepuce, frenulum), urethra, Skene's and greater (Bartholin's) and lesser vestibular glands, perineum, and anus. We are looking for ulcers, plaques, fissures, excoriations, and erythema (diffuse or focal) that could be associated with a specific cause of vulvar pain. We pay particular attention to the vestibular region from four to eight o'clock along the posterior fourchette, which is a common site of redness in women with localized vulvar pain syndrome, although absence of erythema does not exclude the diagnosis, as many patients with vulvodynia have a normal-appearing vestibule. If necessary, we perform tissue biopsy to exclude vulvar dermatoses (eg, lichen sclerosus, psoriasis, lichen planus, atopic dermatitis [eczema], and lichen simplex chronicus) or neoplasm (eg, Paget disease, carcinoma in situ, and squamous cell carcinoma). Lesions suspicious for herpes infection are tested for herpes simplex virus. Episiotomy or surgical scars are inspected for areas of granulation. While use of a colposcope can aid visualization, we avoid using acetic acid because it is typically very painful.
Palpation — The cardinal sign of localized vulvar pain syndrome is significant tenderness upon point-pressure testing of the vulvar vestibule. The vulvar vestibule is located between the hymenal ring and Hart's line on the medial border of the labia minora (picture 1). This area represents the transition between the darker keratinized skin of the labia minora and the lighter epithelium containing openings to the urethra, greater (Bartholin) and lesser vestibular glands, as well as Skene's glands.
We perform point-pressure testing with a cotton-tipped applicator in a circumferential or "paint brush" pattern around the vestibule (eg, similar to a clock face) to map the location and severity of vestibular pain (figure 2). Women with localized, provoked vulvodynia will have pain out of proportion to the pressure from the touch of the cotton swab, particularly in the areas between four and eight o'clock. We record the woman's pain level (mild, moderate, or severe) at each touch point around the vestibule.
Next, in women who are able to tolerate vaginal palpation, we check for tenderness of the vagina, bladder, and pelvic floor muscles and for trigger points. During palpation, we ask the patient to quantify any pain sensation as mild, moderate, or severe. We begin the examination by placing a single digit into the woman's vagina. Next, we ask the patient to abduct her thigh against the opposite hand so that we can palpate the obturator internus muscle. Then we palpate the levator ani bilaterally, the subtrigonal region, and along the posterior fourchette. Tenderness of the pelvic floor muscles or bladder trigone can represent vaginismus, myofascial pain, or interstitial cystitis. Lastly, a bimanual examination is performed with either one or two digits within the vagina to assess the contour of the uterus, adnexa, and uterosacral ligaments and exclude causes of pelvic pain such as uterine fibroids, ovarian cysts, and endometriotic nodules. A cross-sectional analysis of 202 women in the United States National Vulvodynia Registry reported higher pelvic floor muscle pain scores may represent different subgroups of provoked vestibulodynia compared with mucosal pain scores elicited from a cotton-swab test, including low arousal and sexual function and satisfaction scores [78].
Speculum examination — A speculum examination is performed with the smallest speculum that allows adequate visualization of the vaginal walls and cervix. We assess for vaginal pallor and loss of rugae suggestive of vaginal atrophy or genitourinary syndrome of menopause. If abnormal vaginal discharge is present, we collect specimens for microscopy and diagnostic testing. (See "Genitourinary syndrome of menopause (vulvovaginal atrophy): Clinical manifestations and diagnosis" and 'Laboratory' below.)
Laboratory — Localized vulvar pain syndrome does not cause laboratory abnormalities. Laboratory evaluation is done to exclude other causes for the patient's symptoms. For example, if abnormal vaginal or cervical discharge is present, we perform microscopy to assess for common causes of vaginal infection (bacterial vaginosis, candida vulvovaginitis) and test for gonorrhea, chlamydia, and trichomoniasis. Women with pelvic pain commonly have a urinalysis or urine culture performed to exclude infection. Vulvar lesions are biopsied and examined to exclude inflammatory or neoplastic processes (eg, lichen sclerosus, squamous cell carcinoma). However, biopsy is not required to diagnose localized vulvar pain syndrome because the syndrome does not have pathognomonic histological features. (See "Bacterial vaginosis: Clinical manifestations and diagnosis" and "Candida vulvovaginitis: Clinical manifestations and diagnosis" and "Clinical manifestations and diagnosis of Neisseria gonorrhoeae infection in adults and adolescents" and "Clinical manifestations and diagnosis of Chlamydia trachomatis infections" and "Trichomoniasis: Clinical manifestations and diagnosis".)
Imaging — Localized vulvar pain syndrome does not cause imaging abnormalities. Imaging techniques can be useful to identify other causes of pain. As an example, most women with pelvic pain undergo transvaginal ultrasound to assess for uterine fibroids or ovarian cysts. Patients with symptoms or risk factors for kidney stones may benefit from imaging to exclude this diagnosis.
●(See "Chronic pelvic pain in adult females: Evaluation".)
DIAGNOSIS — Localized vulvar pain syndrome is a clinical diagnosis based upon physical examination confirming significant pain during point-pressure testing of the vulvar vestibule with a cotton-tipped applicator (figure 2) in women who have had vestibular pain for at least three months and have no other identifiable cause. (See 'Diagnostic criteria' above.)
Asymptomatic women can also have a positive response to cotton-swab testing and cannot be considered as having vulvodynia based on this testing alone [79]. Supportive history includes description of a burning pain, inability to have vaginal sexual contact or use a tampon, and interference with relationships and routine activities.
COEXISTING PAIN SYNDROMES — Other pain syndromes appear to be more common in some women with localized vulvar pain syndrome. In a study of over 1900 women, vulvodynia was associated with fibromyalgia, interstitial cystitis, and irritable bowel syndrome [80]. Similarly, in a study of 122 women with vulvodynia, 80 percent screened positive for interstitial cystitis [81]. It is not known if localized vulvar pain syndrome represents referred pain from these other sites, different manifestations of the same underlying etiology, or part of a more broad multidimensional etiology. As an example, in a questionnaire study of 137 women with localized vulvar pain syndrome, nearly 80 percent also reported orofacial pain (OFP) symptoms [82]. Additionally, when compared with women who had no OFP symptoms, women with symptoms reported higher levels of anxiety, somatization, and psychologic distress.
Based on the above studies, we screen women suspected of having localized vulvar pain syndrome for additional pain syndromes, particularly interstitial cystitis. (See "Interstitial cystitis/bladder pain syndrome: Clinical features and diagnosis" and "Clinical manifestations and diagnosis of fibromyalgia in adults" and "Clinical manifestations and diagnosis of irritable bowel syndrome in adults".)
TREATMENT — Treatment of vulvodynia, including localized vulvar pain syndrome, is reviewed in detail separately. (See "Vulvar pain of unknown cause (vulvodynia): Treatment".)
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Female pelvic pain".)
SUMMARY AND RECOMMENDATIONS
●Terminology – Localized vulvar pain syndrome is a vulvodynia involving the vulvar vestibule and possibly the clitoris. In contrast to vulvar pain disorders due to a specific cause, localized vulvar pain syndrome has no identifiable cause. (See 'Terminology' above.)
●Pathogenesis – The etiology of localized vulvar pain syndrome is not fully known and is likely multifactorial. The leading hypothesis is that an inciting event, such as infection or trauma, initiates a local inflammatory response. In susceptible women, the inflammatory response causes the proliferation of nerve fibers in the vestibular tissue and lowered pain thresholds, which can then lead to central sensitization of pain. (See 'Pathogenesis and likely contributors' above.)
Specific contributors to localized vulvar pain syndrome include infectious, inflammatory, neurologic, genetic, allergic, hormonal, myofascial, and psychological processes. While psychological dysfunction does not appear to cause vulvar or vestibular pain, the chronic pain of vulvodynia can lead to secondary psychological and mental health disorders. (See 'Pathogenesis and likely contributors' above.)
●Diagnostic criteria – Diagnostic criteria for localized vulvar pain syndrome include:
•Pain localized to the vulvar vestibule and possibly the clitoris
•Absence of identifiable cause
•Duration of at least three months
•Pain elicited with pressure-point testing
Symptom onset can occur with initial vestibular contact (primary) or develop after a period of normal function (secondary). Symptom timing after vestibular contact can be immediate or delayed and symptom pattern can be intermittent, persistent (nonrelapsing), or constant (continuous). Additionally, some women have both localized vulvar pain syndrome and an identifiable vulvar pain disorder with a known, reversible cause. (See 'Diagnostic criteria' above.)
●Clinical presentation – Women with localized vulvar pain syndrome present with significant pain upon contact with the vulvar vestibule (picture 1). Common complaints include pain with vaginal intercourse, tampon insertion, tight clothing, prolonged sitting, biking, or other sports. (See 'Clinical presentation' above.)
●Diagnostic evaluation – The diagnostic evaluation consists of a detailed history and physical examination that includes focal pressure-point testing of the vulvar vestibule. One purpose of the evaluation is to identify potential specific causes of the woman's pain, as localized vulvar pain syndrome is a diagnosis of exclusion. However, the diagnostic process can be confusing as vulvar pain from a specific cause can coexist with localized vulvar pain syndrome. (See 'Diagnostic evaluation' above.)
•History – The history includes a comprehensive review of systems, detailed gynecologic history, and vulvar pain history to characterize the patient’s pain and identify potential specific causes of vulvar pain (table 1). Women with localized vulvar pain syndrome can consistently localize and rate their pain, which is commonly described as a burning sensation. (See 'History' above.)
•Physical examination and findings – The cardinal sign of localized vulvar pain syndrome is significant tenderness upon point-pressure testing of the vulvar vestibule. The pain is mapped by location and severity with a cotton-tipped applicator (figure 2). Visual inspection, vaginal and bimanual examinations, and speculum examination are also performed to identify other possible causes of pain. (See 'Physical examination' above.)
•Laboratory and imaging –Localized vulvar pain syndrome does not cause laboratory or imaging abnormalities. However, laboratory tests such as microscopy; testing for gonorrhea, chlamydia, and trichomonas; and urinalysis or urine culture can be useful to exclude infectious causes. Similarly, pelvic ultrasound is commonly performed as part of the evaluation for pelvic pain. (See 'Laboratory' above and 'Imaging' above.)
●Diagnosis – Localized vulvar pain syndrome is a clinical diagnosis based upon physical examination confirming significant pain during point-pressure testing of the vulvar vestibule (figure 2). Supportive history includes description of a burning pain, inability to use have vaginal sexual contact or use a tampon, and interference with relationships and routine activities. (See 'Diagnosis' above.)
●Coexisting pain syndromes – Other pain syndromes appear to be more common in some women with localized vulvar pain syndrome. It is not known if localized vulvar pain syndrome represents referred pain from these sites or if these syndromes are different manifestations of the same underlying etiology. We screen women suspected of having localized vulvar pain syndrome for additional pain syndromes, particularly interstitial cystitis. (See 'Coexisting pain syndromes' above.)
ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Elizabeth Gunther Stewart, MD, who contributed to an earlier version of this topic review.
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