Camilla Cracchiolo, RN

Finding a physician who can treat vulvodynia;

New treatments for neurologic vulvodynia, including capsaicin, carbamazepine (Tegretol), and       neurotonin;

What are vulvodynia and Vulvar Vestibulitis Syndrome?

How is vulvodynia/vestibulitis diagnosed?

Finding a physician who can treat vulvar pain

Organizations and resources for women with       vulvodynia.

1. What are vulvodynia and Vulvar Vestibulitis Syndrome?

Vulvodynia is a medical term that means "painful vulva". The term can cover a wide variety of vulvar pain syndromes, including various infections and skin disorders.

Vulvar pain syndromes have been written about in medical books since at least the late 1800s. In 1889, Dr. A.J.C. Skene wrote a "Treatise on the Diseases of Women" wherein he described a disorder that was characterized by an "excessive sensitivity" of the vulva. He stated that itching was absent, but when "the examining finger comes in contact with the hyperesthetic part, the patient complains of pain which is sometimes so great as to cause her to cry out". He treated this condition by surgically removing the affected area. However, he noted that this provided only temporary relief.

In 1928, Dr. H.A. Kelly wrote about a condition characterized by "exquisitely sensitive deep-red spots on the mucosa of the hymenal ring" as a frequent source of painful intercourse. However, this condition appears to have then been ignored in medical journals until the early 1980s, when Dr. Edward Friedrich began reporting on it. 

 Since 1983, more than 80 studies have been reported in the medical literature. It is unclear whether this is because this illness is increasing in incidence, or whether physicians are now simply recognizing the symptoms of vulvodynia in their patients due to being better informed. It's possible that both things are happening together.

Today, the term "vulvodynia" is frequently used to refer two conditions:

VVS is an inflammation of the vestibule, or opening to the vagina and the tissues immediately around the vaginal opening. This condition is also sometimes called "vestibular adenitis".The classic description of VVS involves redness of the vulvar vestibule, especially with small red spots; pain with intercourse or tampon insertion and stinging pain when urinating.

This is also called "essential vulvodynia", "pudendal neuralgia" or "dysthetic vulvodynia". The classic description of pudendal neuralgia involves a more or less constant itching or tingling sensation in the vulva, ranging from mild to excruciating pain of the entire vulva. Pudendal neuralgia is probably due to compression or degeneration of the pudendal nerve, one of the main nerves that relays sensation to and from the genitals. This condition can also result from a spinal injury, or a tumor or cyst in the spine. Trauma during childbirth can also cause vulvodynia. In many cases, the exact cause remains unknown.

Israeli researchers suggest there is another subdivision of vulvodynia: vestibulodynia. They believe that vestibulodynia is a unique syndrome that affects women who are older than those who have vestibulitis alone and is associated with the presence of human papillomavirus (HPV), painful urination, and a higher failure rate for surgical treatment than that for vestibulitis.

 However, this subdivision is not universally accepted among researchers or treating physicians.

Also complicating terminology is the fact that it is possible to have neurologic pain of the vestibule, and for a problem which began as an inflammation (VVS) to turn into a long standing pain disorder with nerve involvement.

Because of this, some physicians regard the division between VVS and "essential vulvodynia" as artificial and arbitrary. They think that vulvodynia should be classified as a subset of urinary and genital pain disorders or "painful bladder" syndromes. These syndromes include vulvodynia, urethral syndrome, interstitial cystitis, prostatitis and prostadynia.

There may be some merit to this point of view. 

 Vestibulitis and essential vulvodynia often occur in combination with inflammatory problems of the urinary tract such as interstitial cystitis or urethral syndrome. This is not surprising, since the lining of both vagina and bladder arise from the same tissue during fetal development; thus when one becomes inflamed, the inflammation may spread easily to the adjoining areas. Certainly, it is obvious even to the untrained observer that there is no line demarcating the urethral tissues from the inner lips of the vulva or from the vaginal lining, perineum and mucous membranes of the rectum.

Vulvodynia can range from mild to severe. Usually, a burning or stinging sensation is noted during intercourse or when tampons are inserted; upon touching the area with a cotton swab, pain is felt when the hymen and inner vaginal lips are touched. There may or may not be visible redness and swelling. With vulvodynia of neurologic origin, the pain tends to be more or less constant and more diffused in area. In severe cases, the pain can be agonizing.The clitoris can become involved, becoming painful or hypersensitive and there may shooting pains from the clitoris up the abdomen. With severe VVS, much of the vulva can be reddened, swollen and very inflamed.

Often, there is hypersensitivity along the edges of the inner vaginal lips and the pain can be so severe that it makes walking difficult. A constant itching or stinging sensation in the grooves between the large and small vaginal lips is commonly reported; some women cannot stand to wear underwear for this reason, because the slightest touch to the area results in excruciating pain. Other signs include pain or discomfort upon touching the pubic hair; a feeling of pain or discomfort all over the vulva; sensations of "parchedness" or drying, and "drawing" sensations, either all over the vulvar skin or only in certain spots. These sensations may extend to the rectal area or the skin of the perineum. Urination can become very painful and many women report symptoms that may seem more consistent with a urinary tract infection, such as frequent or painful urination. 

Very few studies have been done that give any information about the incidence of this disease or which women are most likely to get it.

Women of all ages, from adolescence through post-menopause, can develop vulvodynia. Although no one knows exactly how many women suffer from vulvodynia, the Vulvar Pain Foundation estimates that between 100,000 and 150,000 women in the US alone suffer from this condition. 

 The Vulvar Pain Foundation also reports that they have identified cases in pre-pubescent children; however this remains to be studied further.

Dr. Martha F. Goetch, of Good Samaritan Hospital in Portland, OR, attempted to find out what percentage of patients in a standard OB/GYN practice have vulvar pain and what the variation in normal vulvar sensation is. Dr. Goetch tested 210 patients over a six month period of time by questioning them for symptoms and then administering a "swab test". 78 women (37%) showed some signs of vulvar tenderness and 31 (15%) fit the clinical definition for Vulvar Vestibulitis Syndrome. These women were then given a questionnaire to see if any common characteristics could be identified. A total of 50% had long-standing pain, most since their teenage years. Their symptoms did not suggest any cyclical pattern and, interestingly, 32% had a female relative who either had pain with intercourse or who found tampons painful. This raises the possibility that some genetic predisposition for VVS exists. 

 Dr. Stanley Marinoff, of the Center for Vulvovaginal Disorders in Washington, D.C., states in an interview in the NVA News that he has identified seven pairs of sisters and two mother-daughter pairs with vulvodynia, but that this needs more study. 

A study done in London, England indicated that most of the women affected by this were Caucasian, most likely to be in their thirties and middle-to-upper class. 

 However, this is also the group of patients most likely to be well educated and to demand adequate treatment from their physicians. Another study at Wayne State University in Detroit suggests that vulvodynia patients are overwhelmingly white, which may be of significance since the Wayne State teaching hospitals and clinics are located in a very poor area of the city and their patient population is primarily African-American. Interestingly, this same study also found that most women with vulvodynia had never had children. 

 More prevalence studies looking specifically at women of other races and income levels need to be done. It may prove that, like certain other disorders such as Chronic Fatigue Syndrome, vulvodynia is common in other ethnic and income groups who lack the resources to obtain proper diagnosis.

That's the big question that researchers are trying to answer: right now, no one knows. It may have one or several different causes.

Factors associated with pudendal neuralgia:

Most cases of pudendal neuralgia probably originate from damage to the nerves innervating the genitals. It is well known that nerve degeneration causes ongoing pain: for example, many people with diabetes, atherosclerosis, herpes infection or HIV have damage to the nerves in the extremities that leads to constant tingling and pain (called "peripheral neuralgia"). The first two conditions damage the blood supply to the nerve, so that parts of the nerve die; herpes and HIV infection cause damage by direct attack of nerve tissue. In either case, it is the injured nerve that causes the pain. One researcher has reported a higher incidence of vulvodynia in women who are infected with HIV, suggesting that a process similar to HIV-induced peripheral neuralgia may be at work. 

 (However, having vulvodynia does NOT mean that therefore you have HIV.)

Other cases of pudendal neuralgia appear to be due to nerves being "pinched" or compressed. A number of factors can contribute to compressed nerves, including narrowing of the opening in the spinal column from whence the nerve originates; obstetrical trauma; tumors or cysts of the spine, and perhaps ongoing muscular tension in the pelvis.

However, neuralgia can originate at any level in the nervous system. Another neurologic pain disorder that resembles vulvodynia is phantom limb pain, which probably involves the brain as well as damage to the nerves of the amputated limb. Tumors in certain parts of the brain can also cause peripheral neuralgia. The most common involvement of the central (as opposed to peripheral) nervous system is probably dysregulation of the pain "filtering" centers high in the spinal cord and brainstem, leading a person to perceive normal sensations as painful.

Nerve compression probably does not contribute a lot to vestibulitis, which appears to be more of an inflammatory condition. It often has a different characteristic pattern of sensation than vulvodynia of neurologic origin. Women who have pudendal neuralgia usually complain of tingling, itching or burning sensations. In fact, the classic presentation of a peripheral neuralgia anywhere in the body is as a spontaneous burning pain, which may be accompanied by shooting or lancing pains. These women do not usually experience more pain when urinating; women with VVS, on the other hand, can find urination to be extremely painful.

However, some women may have both vestibulitis and dysthetic vulvodynia. One theory about vulvodynia believes that an initial episode of inflammation, such as from an infection, can set up an abnormal pain feedback loop. Again, this may be due to dysregulation of pain control mechanisms high in the spinal cord and brain; it could also be the result of chronic pelvic muscular tension. In other words, the woman starts "guarding" or trying to protect herself from the pain by tensing the muscles; then when the inflammation is gone, the tension remains, leading to more pain.

Several authors distinguish between "acute" VVS and "chronic VVS". In the first case, it is often possible to find a precipitating cause such as infection, whereas in the women with chronic cases it is very difficult to determine what had caused it.

Whether there is an association between vestibulitis and HPV infection is one of the controversies about this condition. In one study, 13 women with symptoms of vestibulitis, who also developed white patches when the tender areas were washed with acetic acid and viewed under blue light, had biopsies of the affected areas. The tissue samples were then sent for DNA analysis to see if HPV was present. 11 of the 13 women showed signs of HPV.

 However, other studies suggest that HPV infection is actually rare in VVS. 

 Some researchers have found that HPV infection is often completely without symptoms, and may even completely regress or vanish without treatment. One study found that HPV was present in half of the women who sought treatment for vestibulitis, yet that it had no relation to either the severity of symptoms or of the response to surgical treatment.

 A characteristic skin condition called "papillomatosis" is often found along with HPV: it looks like tny ridges in the skin and often can only be seen with a microscope. This is often seen in women with vulvodynia; however, it can also be seen in people who have inflamed skin without HPV and even in some people who have no symptoms at all. It's important to remember that many women with vestibulitis show no signs of HPV, and the vast majority of women with genital warts will never go on to develop any vulvar pain syndromes.

Other physicians have suggested that VVS may be the result of infection with an unknown virus - in other words, one different from HPV. So far, no novel viruses have been identified in VVS. Researchers at Carmel Medical Center in Haifa, Israel tried to determine if certain common viruses of the herpes family, known to be implicated in neurologic pain, were present in women with vestibulitis. They conducted polymerase chain reaction analysis to determine the presence of viral genes. They found virtually no prevalence of herpes simplex virus or cytomegalovirus among the subjects. However they DID detect HPV in 46 cases (54%). Only one woman of the 25 asymptomatic controls had HPV DNA in the vestibule. They concluded that vulvar vestibulitis is associated with human papilloma virus DNA in more than half of cases.

 However, Marinoff reports that some women do seem to develop vulvodynia in response to infection with herpes virus. He treats these women with acyclovir, an anti-viral drug, to minimize outbreaks.

An association with chronic candida infection has been found in a couple of studies. 

 This is important because women who have VVS have often had many courses of treatment for vaginal yeast with imidazole family anti-fungals. These drugs have generic names that all end in "azole"; clotrimazole (marketed in the US as Gyne-Lotrimin vaginal cream); ketoconazole (Nizoral oral tablets); miconazole (marketed as Monistat vaginal cream), fluconazole (marketed as Diflucan oral tablets). It's not clear whether these drugs actually can cause vulvodynia or if they are just associated with it because VVS is often mistaken for a chronic yeast infection. However, women should take the package labeling for over the counter vaginal anti-fungal creams seriously; if your problem doesn't clear up after the time period allowed, it's time to see a doctor. Physicians should not try course after course of antifungals in their patients: if antifungals and antibiotics don't help after a few trials, referral to a specialist in vulvar pain disorders is probably indicated.

Camilla Cracchiolo, RN

Articles

Vulvodynia and Vulvar Vestibulitis Syndrome FAQ v2.3