Vulvodynia is a chronic disorder of vulval pain. It is often distressing and has consequences for interpersonal and psychological wellbeing.

It should not be confused with pruritus vulvae where itching is the predominant symptom; the burning and pain associated with vulvodynia would make scratching unbearable.

Its cause is unknown, although it is often presumed to be multifactorial. Accurate clinical diagnosis (to enable effective treatment) is vital, although this can prove difficult.

Classification

The term 'vulvodynia' is recommended by the International Society for the Study of Vulvovaginal Disease (ISSVD) to describe any vulval pain - regardless of aetiology.¹ It redefined vulvodynia as vulval discomfort in the absence of gross anatomical or neurological findings. Further classification occurs on the basis of pain that is:

• Generalised versus localised.
• Provoked, unprovoked, or both.

Four subgroups are recognised:²

Vulval vestibulitis syndrome

• This is associated with dyspareunia or pain on insertion of tampons.
• Chemicals, irritants, allergies, laser and cryotherapy have all been implicated as has infection with human papillomavirus.
• There may be focal tenderness at the vestibule, which may be associated with erythema over Skene's and Bartholin's ducts.
• It may be complicated by vaginismus.

Vulval dysaesthesia

• This tends to affect women around or beyond the menopause.
• Vulval or perineal pain is constant.
• There is less dyspareunia and tenderness is more diffuse than with vulval vestibulitis syndrome.
• It seems to represent an alteration in perception.
• The burning pain is rather similar to that with shingles and would seem to be neurological in origin. It can also occur on the trunk.
• Discomfort in the urethra and anus may be present. It is often associated urinary symptoms such as frequency.
• There is an increased innervation of the sensitive area but whether this neural hyperplasia is the cause or result of the disease remains unknown.³

Cyclical vulvovaginitis

• This is probably the most common form.
• Pain tends to be worse in the second part of the menstrual cycle.
• Sexual activity aggravates pain over the next two or three days.
• It is thought to represent a sensitivity to candidal infection.

Vulval dermatoses

• These are sometimes called secondary vulvodynia.
• They can cause pruritus or pain and may be acute or chronic.
• Unlike other forms of vulvodynia there may be more abnormality on inspection, like erythema, erosion or vesicles.
• Amongst the many dermatological conditions that can be involved are psoriasis, tinea cruris, contact dermatitis, lichen simplex chronicus, lichen planus, lichen sclerosus, pemphigus and erythema multiforme.
• Other disorders of the vulva to consider are herpes zoster that is unusual in this area and Behçet's syndrome.
• The anterior two thirds of the labia majora are supplied by L1 and the posterior third by S2, S3 and S4.
• Dermatological conditions may be atypical.

Aetiology

There are many conditions that cause vulval burning and/or pain:⁴

• Irritant dermatitis is common. Irritants include:
  • Soap, panty liners, synthetic underwear.
  • Moistened wipes, deodorants, douches.
  • Lubricants, spermicides.
  • Topical medication.
  • Urine, faeces, excessive vaginal discharge.
• Allergic contact dermatitis, e.g. prescribed topical medication.
• Other causes include:
  • Oestrogen-deficient vulvovaginal atrophy.
  • Recurrent herpes simplex infection, herpes zoster and post-herpetic neuralgia, lichen sclerosus, erosive lichen planus.
  • Behçet's syndrome, cicatricial pemphigoid, Sjögren's syndrome.
  • Vulval intraepithelial neoplasia and carcinoma.⁵

Epidemiology

• Vulval pain has been shown to be a reasonably common problem; a prevalence study in the USA suggested a current rate of 3.8% of vulval pain of at least 6 months' duration in a random sample of women contacted by telephone.⁶
• A study of patients at a gynaecology clinic found 15% had symptoms to fulfil the definition of vulval vestibulitis.⁷ This high figure is thought to represent the special interest of such clinics and prior selection of patients rather than a true population prevalence.
• It can occur in any age group from the 20s to the 60s and beyond. An Australian study suggests that the condition frequently starts early with the highest prevalence found in the under-25s and the average age of onset for primary cases being 19 years.⁸

Risk factors

Unpicking the role of associated 'comorbidities' or 'risk factors' is difficult; multiple pathologies may initiate or exacerbate symptoms or may share a common pathogenesis with vulvodynia.

Common associations include:

• Human papillomavirus in vulval biopsy.⁹
• Chronic use of topical vulval preparations.¹⁰
• Irritable bowel syndrome (IBS) and fibromyalgia.¹¹
• Chronic bladder and vaginal infections.¹¹

On the basis of the association with other chronic pain conditions like IBS and fibromyalgia, the possibility that vulvodynia is a somatisation disorder has been raised.¹²

Psychological or psychosexual problems have commonly been linked to vulvodynia. As always, questions of cause and effect arise, particularly when diagnosis and treatment may have been protracted and unsatisfactory. Links have been made to past sexual abuse but results of such surveys are variable and unreliable.¹³

A genetic predisposition appears possible with family history of dyspareunia or tampon intolerance in a third of patients.

Diagnosis

Frequently, clinicians begin to suspect the diagnosis after treatment for inflammatory and infectious aetiologies has failed. The diagnosis of vulvodynia requires a careful history and confirmatory physical examination. Failure to achieve a satisfactory diagnosis can result in increasing frustration, worsening psychological problems along with phobia about sexually transmitted disease and cancer.

Symptoms

Symptoms may have been present since childhood or the time of first intercourse or may appear de novo after years of comfortable sexual relations. Onset is usually acute and may be attributed to:

• Vaginitis caused by a yeast or bacterial infection.
• A new sexual partner or increased sexual activity.
• A medical procedure such as cryotherapy or laser.

If it continues unchecked the pain becomes chronic. Severity can be mild to disabling and the nature of the pain is described as burning, stinging, irritating or raw. Allodynia (pain elicited by a non-painful stimulus) and hyperparaesthesia (where a stimulus produces much greater pain than would be normally anticipated) are features of neuropathic pain commonly found with vulvodynia.

The pain may be in the general vulval area (generalised), but is most often in the vulval vestibulum (localised). This is the area between the labia minora and the hymenal ring, anteriorly from the frenulum of the clitoris, and posteriorly from the fourchette to the vaginal introitus. The urethra, Skene's glands, Bartholin's glands and the minor vestibular glands are all in that area. Pain may be constant or may come on suddenly when provoked.

Provoking factors include:¹⁴

• Intercourse
• Tampon insertion
• Prolonged sitting
• Wearing tight clothes

Pain typically dissipates gradually so may linger hours to days following intercourse or pelvic examination. History usually fails to uncover sexually transmitted disease or other significant health problems. There may be an element of depression but this can be secondary rather than an underlying cause of the problem.¹⁵

Signs

• Little or nothing abnormal is apparent on inspection.
• A moist, cotton tip applicator can be used to touch the vestibulum lightly in order to 'pain map'.
• There may be a sharp pain most often in the posterior vestibule, anterior vestibule or both.
• Whilst most regard examination as an important part of diagnosis of this condition, self-reported symptoms show good reliability and validity for predicting vulvodynia.¹⁶

Differential diagnosis¹⁴

Alternative causes of dyspareunia at the vaginal introitus include:

• Allergic vulvitis
• Chronic candidal vulvovaginitis
• Lichen planus
• Lichen sclerosus
• Pudendal canal syndrome
• Vaginismus
• Vulval atrophy
• Vulval intraepithelial neoplasia

Investigations

• Investigation should exclude infective and inflammatory conditions.
• The presence of a rash or altered mucosa or skin on examination is not consistent with the diagnosis and should be evaluated more fully, usually with a biopsy.
• Some advocate screening for active candidal vulvovaginitis, with swabs or even colposcopy, in order that, if present, this can be fully treated and not cloud further treatment needed for vulvodynia.¹⁷
• Many women with vulvodynia will carry Candida albicans but eradication does not usually improve a patient's symptoms.

Management²

Vulvodynia has many possible treatments, but very few controlled trials have been performed to verify efficacy of these treatments.¹⁸ Over recent years, a number of specialist clinics have been set up to treat and investigate the condition and its best management.

Medical

Conventional analgesics and narcotics are not helpful in vulvodynia. Instead, medications used in other neuropathic disorders have been borrowed, including:

• Tricyclic antidepressants (TCAs) - frequently used as first-line therapy. Side-effects are common.¹⁹
• Gabapentin - shown to be efficacious in the treatment of unprovoked generalised vulvodynia.^20,21
• Paroxetine and venlafaxine have been used in patients who could not tolerate TCAs.¹⁴

Topical therapies include:

• Soothing agents such as aqueous cream.² Patients should also be advised to avoid irritants such as soap, bubble baths, shower gels, shampoo, special vaginal wipes or douches, etc. in this area. Use a soap substitute and Vaseline® to protect the area when swimming.
• Lidocaine gel or cream (5%) can be used to control symptoms during sexual intercourse (use 10 minutes prior to intercourse and wipe off fully if using a condom) or as a regular adjunct to other treatment.²

Surgical

Approaches include perineoplasty and vestibulectomy:

• The aim is to remove hypersensitive tissue and replace with the advancement of normal vaginal mucosa.
• It is only appropriate for localised disease and tends to be reserved for patients who have had limited success with other therapies.
• Failure rates are higher if pain was present from first intercourse or was constant.²²

Physiotherapy

• Pain in the vulva can cause spasm of the adductor muscles of the thigh and other muscles in that region and physiotherapy can be beneficial.
• Biofeedback training has also been used to improve strength and relaxation of the pelvic floor musculature.
• Devices to make sitting more comfortable may also be helpful.

Psychological therapies

• Cognitive and behavioural therapies have been used successfully to improve reported vulval pain with intercourse (as with many other chronic pain syndromes).²³
• Additional support may be required - including reassurance of the partner.
• Sexual, individual or relationship counselling may also be appropriate.

Complementary and alternative medicine

Where conventional medicine proves unsatisfactory, patients frequently turn to alternative practitioners. Few data are available on which to base recommendations for or against such treatments.

A GP should be able to provide empathy and education. Give the patient the opportunity to discuss her problems. Encourage perseverance with treatment. Refer to a specialist where diagnosis is in doubt or where a patient is unresponsive to treatment.

Complications

A controlled study of women with vulvodynia revealed significantly higher levels of psychological distress in the vulvodynia group within the domains of somatisation, obsessive-compulsive behaviour, depression, anxiety and phobic symptoms, as well as with interpersonal sensitivity hostility and paranoia.²⁴ There was no suggestion of whether this was a contributory factor or the result of the condition. Another showed that 'adverse life experiences, including conflict, are common in women with vulvodynia' and suggested that the pain may have been stress-related.²⁵

Prognosis

The natural history of this disorder is not clear. A long-term questionnaire follow-up study of 234 women (unfortunately with a low response rate reducing its validity) found that a majority (57%) reported at least 50% improvement in their pain score whilst fewer than 2% had deteriorated.²⁶ Pregnancy did not often aggravate symptoms.

Document references

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2. Management of Vulval Conditions, Clinical Effectiveness Group, British Association Sexual Health & HIV (2007)
3. Tympanidis P, Terenghi G, Dowd P; Increased innervation of the vulval vestibule in patients with vulvodynia. Br J Dermatol. 2003 May;148(5):1021-7. [abstract]
4. Lotery HE, McClure N, Galask RP; Vulvodynia. Lancet. 2004 Mar 27;363(9414):1058-60. [abstract]
5. Fischer M, Marsch WC; Vulvodynia: an indicator or even an early symptom of vulvar cancer. Cutis. 2001 Mar;67(3):235-8. [abstract]
6. Arnold LD, Bachmann GA, Rosen R, et al; Assessment of vulvodynia symptoms in a sample of US women: a prevalence survey with a nested case control study. Am J Obstet Gynecol. 2007 Feb;196(2):128.e1-6. [abstract]
7. Goetsch MF; Vulvar vestibulitis: prevalence and historic features in a general gynecologic practice population. Am J Obstet Gynecol. 1991 Jun;164(6 Pt 1):1609-14; discussion 1614-6. [abstract]
8. Jantos M, Burns NR; Vulvodynia. Development of a psychosexual profile. J Reprod Med. 2007 Jan;52(1):63-71. [abstract]
9. Orlandi A, Francesconi A, Angeloni C, et al; Prevalence and genotyping of human papillomavirus infection in women with vulvodynia. Acta Obstet Gynecol Scand. 2007;86(8):1003-10. [abstract]
10. Bachmann GA, Rosen R, Pinn VW, et al; Vulvodynia: a state-of-the-art consensus on definitions, diagnosis and management. J Reprod Med. 2006 Jun;51(6):447-56. [abstract]
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12. Mascherpa F, Bogliatto F, Lynch PJ, et al; Vulvodynia as a possible somatization disorder. More than just an opinion. J Reprod Med. 2007 Feb;52(2):107-10. [abstract]
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17. Pagano R; Value of colposcopy in the diagnosis of candidiasis in patients with vulvodynia. J Reprod Med. 2007 Jan;52(1):31-4. [abstract]
18. Haefner HK, Collins ME, Davis GD, et al; The vulvodynia guideline. J Low Genit Tract Dis. 2005 Jan;9(1):40-51. [abstract]
19. Reed BD, Caron AM, Gorenflo DW, et al; Treatment of vulvodynia with tricyclic antidepressants: efficacy and associated factors. J Low Genit Tract Dis. 2006 Oct;10(4):245-51. [abstract]
20. Harris G, Horowitz B, Borgida A; Evaluation of gabapentin in the treatment of generalized vulvodynia, unprovoked. J Reprod Med. 2007 Feb;52(2):103-6. [abstract]
21. Updike GM, Wiesenfeld HC; Insight into the treatment of vulvar pain: a survey of clinicians. Am J Obstet Gynecol. 2005 Oct;193(4):1404-9. [abstract]
22. Bornstein J, Zarfati D, Goldik Z, et al; Perineoplasty compared with vestibuloplasty for severe vulvar vestibulitis. Br J Obstet Gynaecol. 1995 Aug;102(8):652-5. [abstract]
23. Weijmar Schultz WC, Gianotten WL, van der Meijden WI, et al; Behavioral approach with or without surgical intervention to the vulvar vestibulitis syndrome: a prospective randomized and non-randomized study. J Psychosom Obstet Gynaecol. 1996 Sep;17(3):143-8. [abstract]
24. Wylie K, Hallam-Jones R, Harrington C; Psychological difficulties within a group of patients with vulvodynia. J Psychosom Obstet Gynaecol. 2004 Sep-Dec;25(3-4):257-65. [abstract]
25. Plante AF, Kamm MA; Life events in patients with vulvodynia. BJOG. 2008 Mar;115(4):509-14. [abstract]
26. Reed BD, Haefner HK, Cantor L; Vulvar dysesthesia (vulvodynia). A follow-up study. J Reprod Med. 2003 Jun;48(6):409-16. [abstract]

Acknowledgements