Obscure diagnosis - vulvodynia
Our series of uncommon conditions continues with obstetrics and gynaecological consultant Mr Ashfaq Khan discussing vulvodynia.
Joanna, a 31-year-old nulliparous woman, complains of excruciating pain when her partner touches her vulva during intercourse. About 18 months ago, she first noticed vulval soreness while inserting vaginal tampons during her menstruation. She stopped using tampons 12 months ago and received repeated treatment for suspected candidiasis. Her symptoms did not improve and progressed to generalised vulval soreness later on.
Vulval pain was first mentioned in medical literature in 1889. Unfortunately, this condition is still widely unrecognised by general and specialist doctors.
Vulvodynia is characterised by unexplained chronic discomfort in the vulval region lasting for more than three months. The International Society for the Study of Vulvovaginal Diseases (ISSVD) adopted the term ‘vulvodynia’ in 1983, defining it as ‘chronic vulval discomfort that is characterised by the complaint of burning, stinging, irritation, or rawness’ in the absence of skin disease or infection. It is difficult to predict the true incidence of this widely unreported and unrecognised condition, but a small, recent population-based study reported 3.5% incidence of vulvodynia in the USA.
The term ‘vulval vestibulitis’ is no longer used because inflammation is not always associated with this disorder. Dysaesthetic vulvodynia and vestibulitis are also obsolete terms.
The exact aetiology of vulvodynia is unknown. It could be combinations of neurological, immunological, or embryologic developmental factors.
Classification of vulval pain
Table 1: International Society for the Study of Vulvovaginal Diseases (ISSVD) classification of vulval pain
A. Vulval pain related to a specific disorder
- Infectious (candidiasis, herpes, etc.)
- Inflammatory (lichen planus, lichen sclerosus, immunobullous disorders, etc.)
- Neoplastic (Paget’s disease, squamous cell carcinoma, etc.)
- Neurological (herpes neuralgia, spinal nerve compression, etc.)
- Provoked (sexual, nonsexual, or both)
- Mixed (provoked and unprovoked)
2. Localised (vestibulodynia, previously known as vulval vestibulitis, clitorodynia, hemivulvodynia, etc.)
- Provoked (sexual, nonsexual, or both)
- Mixed (provoked and unprovoked)
How do the patients present?
The pain often is described as ‘burning’, but it may be irritating, sharp, prickly, or occasionally pruritic, and it can be mild to severe. Symptoms of vulvodynia may have been present since childhood or the time of first intercourse, or they may appear after several years of painless sex.
Generalised vulvodynia can present as generally dull pain with episodes of shooting pain into the pelvis, inner upper thighs or even in the rectal area. Patients may complain of pain from the lightest touch of underwear. This type of vulvodynia is more common in elderly women. Sexually active women may deny any sort of dyspareunia in case of generalised unprovoked vulvodynia.
Nine out of 10 women with localised (provoked) vulvodynia may complain about superficial dyspareunia. They often report hours to days of discomfort after intercourse. Almost half of them may feel discomfort or vulval pain even by touching the genitalia. Other exacerbating factors are insertion of vaginal tampons, horse or bicycle riding, or wearing tight trousers. Some unfortunate women get vulvodynia after sitting for long periods of time on a chair or a couch. On the other hand, patients with generalised vulvodynia rarely give a history of the initiating event.
Vulvodynia patients, especially those who suffer from the generalised unprovoked variety, frequently have other chronic pain problems like lower back pain, fibromyalgia, and temporo-mandibular joint dysfunction. It is not unusual for treated or untreated localised provoked vulvodynia to present as generalised unprovoked vulvodynia later on.
Can depression cause vulvodynia?
This is difficult to answer, as we know that most chronic pain in these cases is associated with depression. In the past, it was believed that the pain of vulvodynia was due to psychological issues. However, recent data indicate that women with vulvodynia are psychologically comparable to women without the disorder and are no more likely to have been abused, although patients with vulvodynia could be more anxious than the patients with other vulval diseases.
How to diagnose vulvodynia
Vulvodynia is a diagnosis of exclusion, recognising pain with no other identified cause. It is important to take a thorough history, including information about the onset and character of the pain, provoking and relieving factors, medical evaluations to date, attempted treatments and their effects on the pain experienced by the patient. Most importantly, a systematic assessment of the vulva should be performed (see table 2 below). Occasionally, the patient may not be aware that the sensitivity is in the area of the introitus, and she may describe the pain as deeper in the vagina or pelvis.
Table 2: Steps of assessment for vulvodynia
Step 1 : Visual inspection (exclude obvious skin lesions)
Step 2 : Q-tip test (to diagnose localised vestibulodynia)
Step 3 : Neurosensory examination
Step 4: Pelvic-floor muscle exam
Step 5: Evaluation of comorbidity and contributing factors
A cotton swab is used to gently indent – approximately 5mm – several locations on the labia, introitus, and hymenal remnants. This pressure will elicit discomfort in almost all women with vulvodynia, as the posterior introitus and the posterior hymenal remnants are the most common sites of increased sensitivity. The patient can be asked to give a pain score, which can be used to monitor the prognosis of treatment. Although some women have spontaneous pain that may not be provoked with a cotton swab, a lack of sensitivity in all of these areas is unusual among women with provocable pain. The vagina should be examined to exclude any infection and inflammation, using swab tests, wet mounts, and vaginal pH tests as required.
Vulvovaginitis: A persistent candidial infection with thick vaginal discharge. Pruritus is more of a common symptom than pain. Both the vulva and vagina may show erythema and oedema.
Vulval atrophy: Usually found in post-menopausal women. Presents as a pale, thinning mucosa, with possible tears or petechiae.
Vaginismus: Found with anxious and young women, presenting as pelvic floor muscle spasms and accentuated with examinations.
Vulvar intraepithelial neoplasia (VIN): Patient may be asymptomatic or have pruritus. Always associated with changes in colour or architecture of the vulval skin. Usually features white or multicoloured elevated lesions.
Lichens sclerosus: Vulvar thinning, whitening, and wrinkling of the vulval skin, with a disappearance of the labia and clitoral hood. Pruritus can be severe.
Allergic vulvitis (eczema): Presents as pruritus, irritation, and burning. History should be consistent with allergen exposure, with a lack of any infectious cause.
How to treat this pain?
There is a paucity of information regarding the effectiveness of various treatment options for vulvodynia, and many of the commonly recommended treatments have not been systematically studied in randomised controlled trials (RCTs). Significant symptom relief can be achieved by improving the general care of the vulva’s health. Additional help from medication, with or without physiotherapy (including pelvic floor exercises and trigger point therapy), as well as psychosexual counselling, can improve it further. Biofeedback and physical therapy have been used to reverse changes in the pelvic floor musculature and to help women regain control of the muscles, including improving strength and relaxation. Surgical treatment has a limited role in the management of vulvodynia, and should be considered as a last resort.
Here is some general advice about vulval care that can minimise the irritation:
- Wearing 100% cotton underwear, and no underwear at night
- Avoid inserting vaginal tampons
- Clean the vulva with only water or emollients. Use mild and simple soaps for bathing, but do not apply any to the vulva
- Avoid douching and vulvar irritants such as perfumes, dyes, shampoos, and detergents
- It is better to take showers or a quick bath than a long, hot baths
- Pat the area dry after bathing, and applying a preservative-free emollient
- Avoid the use of hair dryers on the vulvar area
- Rinse and pat dry the vulva after urination
- Use adequate lubrication for intercourse
- Applying cool gel packs to the vulvar area
A Committee Opinion from the American College of Obstetricians and Gynaecologists on vulvodynia2 stated that commonly-prescribed topical medications include a variety of local anaesthetics, which can be applied immediately before intercourse or in extended use, oestrogen cream, and tricyclic antidepressants compounded into topical form. Guidelines on vulvodynia from the British Society for the Study of Vulval Disease3 state that a trial of a local anaesthetic agent may be considered in all vulvodynia subsets (Grade of recommendation C; evidence level IV). Topical lidocaine acts locally as a peripheral nerve sodium channel blocker, modulating pain and neurogenic inflammation. Table 3 (below) shows various medications used for the treatment of vulvodynia.
Drugs used for neuro-sensitisation
- Lidocaine and other sodium channel blockers
- Topical oestrogen
- Topical opioids
- Topical NSAIDs
- Other topicals, such as amitriptyline and gabapentin
- Antidepressants, like TCAs, selective serotonin reuptake inhibitors (SSRIs), and SNRI–duloxetine
- Anticonvulsants, including gabapentin, pregabalin, levetiracetam, and lamotrigine
- Opioids, such as morphine, oxycodone, and tramadol
- Other meds, including mexiletine, cannabinoids, bot
Tricyclic antidepressants (TCAs) often are used as a first-line therapy. Many patients tolerate TCAs well after approximately one week of use, but prolonged fatigue, constipation, and weight gain may lead to a requirement to change the type of medication or dosage. Secondary amine TCAs such as nortriptyline or desipramine are preferred over tertiary amine TCAs like amitriptyline and Imipramine, because of better patient tolerance in light of similar therapeutic efficacy. Although SSRIs are not commonly considered effective for the treatment of neuropathic pain, many patients who cannot tolerate TCAs have responded well to SSRIs.
Major side-effects for gabapentin and pregabalin include sedation, dizziness, and peripheral edema. Gabapentin is increased incrementally. Dose titration may begin with 300mg, advancing by short intervals to 900mg, and then more slowly to a higher dose. There are few drug-to-drug interactions with gabapentin. In successful cases, gabapentin is well-tolerated over extended periods of time. However, pregabalin may carry some advantages over gabapentin, as it can be administered bd, may be faster acting, and may have some intrinsic anxiolytic properties. However, a major disadvantage of pregabilin is cost.
Complete or partial vestibulectomy may have a place in the treatment of localised provoked vulvodynia. Nevertheless, most experts do not recommend surgery for women with this disorder unless symptoms are severe and other treatments have not been effective. As vulvodynia may be a transient condition in a substantial percentage of women, reserving surgical approaches when possible seems prudent.
- Vaginal lubricants can help during intercourse. These are like vaginal mucus, and last longer than conventional lubricants. Complementary treatments are widely used by women with vulval pain.
- Aloe vera gel, calendula, rescue cream and Hypercal creams are alternative treatments. Aqueous cream is a perfume-free emollient, which soothes and rehydrates the skin. It can be used as a soap substitute.
- Emulsifying ointments can be used for washing or as a moisturiser. Some patients may find it too thick and may feel reluctant using them, but it can be thinned down with boiling water. Emulsifying ointments, or alternatively, Epaderm and Cetraben cream are effective substitutes.
- Oatmeal sitz baths can be an alternative treatment in severe vulvodynia. Place one sachet in the bath and bathe for 20 minutes. This can be repeated up to four times a day.
- Hypnotherapy and aromatherapy
- Physical therapy and biofeedback can also be helpful. Physical therapy may include exercise, education, or manual therapies, such as massage, joint mobilisation, or soft-tissue mobilisation. Other methods of physical therapy can involve ultrasound, electrical stimulation, or biofeedback techniques.
- Vulval pain can have an emotional or psychological aspect, and some women benefit from psychological counselling, sex therapy, or both. Sex therapy can provide education and information for individuals or couples. Psychological treatment can provide techniques for relaxation or coping with pain or an opportunity to explore other conditions that may relate to the pain.
The natural history of vulvodynia is still not clear. Many women have had this disorder for years before the condition is diagnosed. It causes serious distress to many patients and frustration among the general and specialist practitioners. Vulvodynia occurs at any age, and GPs must consider it as a possible diagnosis when any vulval symptoms are present.
1. Moyal-Barracco M, Lynch PJ. 2003 ISSVD terminology and classification of vulvodynia: a historical perspective. Journal of Reproductive Medicine. 2004; 49(10): 772-7
2. Haefner HK, Collins ME, Davis GD et al. The vulvodynia guideline. J Low Genit Tract Dis 2005; 9: 40–51
3. Nunns D, Mandal D, Byrne M, McLelland J, Rani R, Cullimore J, Bansal D,Brackenbury F, Kirtschig G, Wier M;British Society for the Study of Vulval Disease (BSSVD) Guideline Group. Guidelines for the management of vulvodynia.Br J Dermatol. 2010 Jun; 162(6): 1180-5