BASICS
DESCRIPTION
- Vulvar pain lasting 3 months or more. Occurs in the absence of relevant visible findings, relevant lab abnormalities, or a clinically identifiable neurologic disorder
- Classification by ISSVD is based on whether pain is generalized or localized and whether it is provoked (by physical contact), spontaneous, or mixed.
- Generalized: involvement of majority of the vulva; usually persistent or spontaneous pain
- Localized: severe pain of certain vulvar areas such as the vestibule (formerly known as vestibulodynia), usually provoked with touch or attempted vaginal entry; thought to be the leading cause of painful intercourse among premenopausal women
- Primary: introital dyspareunia from first episode of intercourse or first insertion of tampon or vaginal speculum
- Secondary: introital dyspareunia developing after a period of painless intercourse, tampon use, or speculum exams
EPIDEMIOLOGY
- Most women diagnosed between age 20 and 80 years
- Nearly half of woman opt not to seek treatment (1).
- Patients are psychologically comparable with asymptomatic controls and have similar marital satisfaction.
Incidence
- Recent retrospective study estimates annual rate of new onset vulvodynia to be 1.8% (2).
- Evidence indicates lifetime cumulative incidence approaches 15%, suggesting nearly 14 million U.S. women will experience persistent vulvar discomfort at some point in their lives (3).
Prevalence
- Reports between 8.3% and 16%; non " clinical-based studies approximate a prevalence of 7% with validation by exam (1).
- Studies show Hispanics are 80% more likely to present with vulvar pain compared with Caucasians and African Americans.
ETIOLOGY AND PATHOPHYSIOLOGY
- Vulvodynia is likely to be neuropathically mediated:
- Allodynia and hyperalgesia are thought to result from neurogenic inflammation, leading to sensitization of primary afferents by inflammatory peptides, prostaglandins, and cytokines. Impulses transmitted to the CNS, where reinforcing signals sustain pain loop
- In recent investigations of vulvar biopsy specimens, increased neuronal proliferation and branching in vulvar tissue are evident when compared with tissue of asymptomatic women.
- Pelvic floor pathology also should be considered: In one study, the vulvodynia group showed an increase in pelvic floor hypertonicity at the superficial muscle layer, less vaginal muscle strength with contraction, and decreased relaxation of pelvic floor muscles after contraction (3).
- No cause of vulvodynia has been established. It is most likely a neuropathic pain caused by a combination of the following:
- Recurrent vulvovaginal candidiasis or other infections
- Immune-mediated chronic neuro-inflammatory process within vulvar tissues
- Chemical exposure (trichloroacetic acid) or physical trauma
- Reduced estrogen receptor expression/changes in estrogen concentration
- CNS etiology, similar to other regional pain syndromes
RISK FACTORS
- Vulvovaginal infections, specifically candidiasis. Unclear if infection, treatment, or underlying hypersensitivity is the cause (1). Multiple infections compound this risk.
- Hormonal factors: Controversial evidence proposes increased risk with use of oral contraception pills (OCPs); early age at first use of OCPs and longer duration of use has been associated with increased risk.
- Pelvic floor dysfunction: Increased instability of pelvic floor muscles may perpetuate vulvar tissue inflammation, leading to vascular changes and histamine release.
- Comorbid interstitial cystitis and painful bladder syndrome; potentially related to common embryologic origin of structures
- Abuse: increased risk of vulvodynia if childhood had physical or sexual abuse by a primary family member; causal relationship remains unclear (3).
- Depression and anxiety (1)
- Other neuropathic disorders, including regional pain syndrome
Genetics
Proposed genetic deficiency impairing one 's ability to stop the inflammatory response triggered by infection or chemicals; homozygosity of the two alleles of the IL-1 receptor antagonist occurs in 25 " 50% of vestibulodynia patients, compared with <10% in controls (4).
GENERAL PREVENTION
- Wear 100% cotton underwear in the daytime and no underwear to sleep.
- Avoid douching and other vulvar irritants such as perfumes, dyes, and detergents.
- Avoid abrasive activities and tight, synthetic clothing.
- Avoid panty liners.
- Clean the vulva with water only and pat area dry after bathing.
- Avoid use of hair dryers in the vulvar area.
COMMONLY ASSOCIATED CONDITIONS
Higher incidence of chronic pain syndromes associated with vulvodynia, including chronic cystitis, irritable bowel syndrome, fibromyalgia, migraines, depression, endometriosis, low back pain. Women with vulvodynia have a higher incidence of depression and anxiety both preceding and resulting from their symtoms (1).
DIAGNOSIS
- Vulvodynia is a clinical diagnosis and it should be suspected in any women with chronic pain at the introitus and vulva (4)[B].
- Pain should be characterized using a standard measure such as the McGill Pain Questionnaire; duration and nature of the pain should be established. Use physical exam to rule out other causes of vulvovaginal pain. Negative fungal culture, along with relevant history and positive cotton swab test, confirm diagnosis.
HISTORY
Adequate sexual, social, and pain history should be taken to assess degree of symptoms. Visual pain scales and pain diaries may be helpful (5)[B]:
- Onset of vulvodynia often sudden and without precedents
- Pain often described as generalized, unprovoked.
- Quality of pain is burning, stinging, irritating, or rawness (1).
- Specifically ask about bowel and bladder habits, history of trauma or abuse, history of infections including herpes, and personal hygiene.
- Specific skin complaints may suggest alternate diagnosis; a history of allergies may suggest vulvar dermatitis.
- Assess for precipitants of vulvar pain: tight garments, bicycle riding, tampon use, prolonged sitting, perfumed or deodorant soaps, douching (1)
- Assess for complaints of dyspareunia:
- Presence of vaginismus (involuntary vaginal muscle spasm), adequate lubrication, anorgasmia, partner problems, abuse
- Psychosexual morbidity significantly higher in patients with vulvodynia; counseling may complement medical interventions.
PHYSICAL EXAM
- Ask patient to show where pain is localized or most painful.
- Mouth and skin exams to assess for lesions suggestive of lichen planus or lichen sclerosus
- Vaginal exam should be done to exclude other causes of vulvovaginal pain, including external inspection; palpation; and single digit, speculum, and bimanual exams:
- The vulva may be erythematous, especially at the vestibule. Discomfort with separation of the labia minora is common.
- Spontaneous or elicited pain at the lower ½; of anterior vaginal wall suggests bladder etiology.
- Bulbocavernosus and anal wink reflexes should be checked to assess for peripheral neuropathy.
DIFFERENTIAL DIAGNOSIS
- Infections: candidiasis, herpes, human papillomavirus (HPV), bacterial vaginosis, trichomoniasis, dermatophytes
- Inflammation: lichen planus, immunobullous disorder, allergic vulvitis, lichen sclerosus, atrophic vaginitis
- Neoplasia: Paget disease, vulvar or vaginal intraepithelial neoplasia, squamous cell carcinoma
- Neurologic/muscular: herpes neuralgia, spinal nerve compression, vaginismus
DIAGNOSTIC TESTS & INTERPRETATION
- Cotton swab or Q-tip test: Vulva tested for localized areas of pain beginning at thighs and continuing medially toward vestibule using the soft end and broken sharp end of the cotton swab. Five distinct positions (2, 4, 6, 8, and 10 o 'clock) surveyed using light palpation. Pain rated on a scale from 0 (none) to 10 (most severe). Posterior introitus and posterior hymenal remnants most common sites of increased sensitivity.
- Test for concurrent vaginismus: Apply pressure with a gloved finger to levator ani and obturator internus muscles to assess for tenderness, pain, or contracture.
Initial Tests (lab, imaging)
- Vaginal pH, wet mount, and yeast culture are recommended to rule out vaginitis.
- Gonorrhea and chlamydia testing done at physician 's discretion
- HPV screening is unnecessary; no association has been identified between HPV and vulvodynia.
Follow-Up Tests & Special Considerations
- Varicella-zoster and herpes simplex virus should be considered if ulcers or vesicular eruptions are present.
- Consider biopsy if concerned for neoplasm or dermatophyte infection or if the patient is resistant to treatment.
Diagnostic Procedures/Other
Colposcopy can be helpful if epidermal abnormalities are present. This should be done with caution because acetic acid worsens vulvar pain.
Test Interpretation
No specific histologic features are associated with vulvodynia, although reactive squamous atypia has been observed. Biopsies are unnecessary for diagnosis. Presence of rash/altered mucosa is not consistent with vulvodynia; this requires further evaluation (5)[C].
TREATMENT
A trial of several medications for at least 3 months is usually needed.
GENERAL MEASURES
Combining treatments should be encouraged when treating women with vulvodynia (5)[C]: Various reports on use of a combination of medical treatments, psychotherapy, and dietary intervention reveal women on these combinations do significantly better compared with those who receive medication only.
MEDICATION
- Oral therapies
- Tricyclic antidepressants (TCAs): first-line treatment for unprovoked vulvodynia (5)[B]; do not stop use abruptly; contraindicated in patients with cardiac abnormalities and those taking MAOIs; fatigue, constipation, sweating, palpitations, and weight gain are most common side effects
- Amitriptyline, nortriptyline: most widely studied; start at 10 mg daily; dose should be titrated to pain control. Average effective dose is 60 mg daily. In one study, a 47% complete response rate was recorded (6)[B]. Nortriptyline may be preferred due to less anticholinergic adverse effects.
- Anticonvulsant therapies:
- Gabapentin: Started at 300 mg daily at HS and increased by 300 mg every 3 days. Maximum recommended dose is 3,600 mg daily divided into 3 doses. Dosing regimen limits popularity.
- Topiramate and lamotrigine have been recommended if other therapies are not effective.
- SSNRIs: not commonly used; however, have been helpful in those who cannot tolerate TCAs
- Venlafaxine or duloxetine have also been used; evidence is limited.
- Opioids/NSAIDs: not consistently helpful in relieving vulvar pain
- Topical therapies
- A trial of local anesthetics may be recommended for all patients who present with vulvodynia. Use judiciously to avoid increased irritation (5)[C].
- Lidocaine 5% ointment: for provoked vestibulodynia; application advised 15 to 20 minutes prior to intercourse. Penile numbness and possible toxicity with ingestion can occur.
- In one study, lidocaine 5% ointment was left in vestibule overnight (average of 8 hours) for a period of 6 to 8 weeks; at follow-up, up to 76% of women reported no discomfort with intercourse (3)[C].
- Cromolyn 4% cream: decreases mast cell degranulation in vulvar tissue; recommended application TID (1)[C]
- Capsaicin 0.025%: decreases in discomfort and increases in frequency of intercourse with 20-minute daily application (3)[B]
- Topical amitriptyline 2% combined with baclofen 2% is helpful in patients with comorbid vaginismus.
- Topical corticosteroids and testosterone creams have not been shown to alleviate symptoms of vulvodynia.
- Gabapentin 3 " 6% ointment (must be compounded)
- Topical nitroglycerin may be helpful but may cause headaches.
- Topical estrogen
- Injectable therapies
- Triamcinolone acetonide: no more than 40 mg should be injected monthly; is best when combined with 0.25 " 0.5% bupivacaine
- Submucosal methylprednisone and lidocaine: reports of up to 68% response rate with weekly injections (5)[B]
- Interferon-α: useful in treatment of vestibulodynia. Side effects (myalgias, fever, malaise) limit its use.
- Botulinum toxin A injectable is being studied.
ISSUES FOR REFERRAL
A team approach is recommended for most effective management. Triage to psychosexual medicine, psychology, partner therapy, and pain management teams should be strongly considered (5)[B].
ADDITIONAL THERAPIES
- Cognitive-behavioral therapy (CBT): One randomized trial revealed that CBT is associated with a 30% decrease in vulvar discomfort with sexual intercourse. CBT is the recommended treatment for patients who present with dyspareunia as a main complaint (4)[B].
- Biofeedback/physical therapy: useful with concomitant vaginismus. Treats both generalized and localized vulvar pain; treatment value for unprovoked pain remains unclear. Most studies report an average of 12- to 16-week treatment time.
- Vaginal dilators
- Surface electromyography (sEMG): efficacious for pelvic floor rehabilitation. Patients are more likely to experience pain-free sexual intercourse after sEMG. Significant reductions seen on pain measures at long-term follow-up.
COMPLEMENTARY & ALTERNATIVE MEDICINE
Acupuncture: Small studies of women with unprovoked vulvodynia who did not respond to conventional treatment reported significant decreases in pain severity with acupuncture; treatment value with provoked pain is unknown (5)[C].
SURGERY/OTHER PROCEDURES
- Surgery may be considered for patients with localized symptoms who have failed to respond to other measures. Not recommended for generalized vulvodynia (5)[B]
- 60 " 80% of women who undergo surgery report a significant reduction in pain symptoms; however, when surveyed, patients prefer behavioral therapies than surgical intervention.
- All patients who are considering surgical intervention should be tested and treated for vaginismus. Vestibulectomy is less successful in this subgroup.
- Surgical approaches
- Local excision: precise localization of painful areas; tissue closed in elliptical fashion
- Total vestibulectomy: Tissue is removed from Skene ducts to perineum. The vagina is then brought down to cover defect.
- Perineoplasty: vestibulectomy plus removal of perineal tissue; incision usually terminated above the anal orifice; reserved for severe cases (4)[B]
ONGOING CARE
PATIENT EDUCATION
Patients should be reassured that this condition is neither infectious, nor does it predispose to cancer (5)[C]. Emphasize self-hygiene. Encourage treatment with home remedies, including ice packs, sitz baths with baking soda, olive oil, and barrier cream to preserve moisture after bathing.
PROGNOSIS
Traditionally viewed as a chronic pain disorder, new evidence of remission has been documented; recent 2-year follow-up study revealed 1 in 10 vulvodynia patients reported remission regardless of treatment (7).
REFERENCES
11 Shah M, Hoffstetter S. Vulvodynia. Obstet Gynecol Clin N Am. 2014;41(3):453 " 464.22 Reed BK, Haefner HK, Sen A, et al. Vulvodynia incidence and remission rates among adult women: a 2-year follow-up study. Obstet Gynecol. 2008;112(2, Pt 1):231 " 237.33 Boardman LA, Stockdale CK. Sexual pain. Clin Obstet Gynecol. 2009;52(4):682 " 690.44 Reed BD. Vulvodynia: diagnosis and management. Am Fam Physician. 2006;73(7):1231 " 1238.55 Nunns D, Mandal D, Byrne M, et al. Guidelines for the management of vulvodynia. Br J Dermatol. 2010;162(6):1180 " 1185.66 Stockdale CK, Lawson HW. 2013 vulvodynia guideline update. J Low Genit Tract Dis. 2014;18(2):93 " 100.77 Davis SN, Bergeron S, Binik YM, et al. Women with provoked vestibulodynia experience clinically significant reductions in pain regardless of treatment: results from a 2-year follow-up study. J Sex Med. 2013;10(12):3080 " 3087.
CODES
ICD10
- N94.819 Vulvodynia, unspecified
- N94.818 Other vulvodynia
- N94.810 Vulvar vestibulitis
ICD9
- 625.70 Vulvodynia, unspecified
- 625.79 Other vulvodynia
- 625.71 Vulvar vestibulitis
SNOMED
- 238968009 vulvodynia (disorder)
- 30833006 vulvar vestibulitis (disorder)
CLINICAL PEARLS
- Vulvodynia is a clinical diagnosis; it should be suspected in any woman with chronic pain at the introitus and vulva.
- A decrease in pain may take weeks to months and may not be complete.
- No single treatment is proven in all women; improvement over time is common even without treatment.