Vulvodynia and Vulvar Vestibulitis Syndrome FAQ v2.3by Camilla Cracchiolo, RN |
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. (1,4) 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.(31) 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. (5,6)) 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.(2,3)
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. (2,3)
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. (9,10) 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. (7) 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. (37)
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. (8) 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. (42) 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.
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. (41) (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.(11,12) However, other studies suggest that HPV infection is actually rare in VVS. (13,14) 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.(26) 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.(27) 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.(36)
An association with chronic candida infection has been found in a couple of studies. (13,23) 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.
One study found that 17% of its subjects had a type of bacteria called ureaplasma in their Bartholin's glands.(13) Another study found chronic inflammation of the Bartholin's glands in women with VVS of more than 5 years duration. The inflammation was identified by microscopic examination. These researchers found that 43% improved with laser treatment of the Bartholin's ducts and many of those who did not respond to laser did respond to surgical treatment. (30)
The Goetch study noted that several of the women studied had recently had a baby and that others were infected with Group B streptococcus.(7)
Two studies have implicated early use of oral contraceptives and an early age of first sexual intercourse as risk factors and suggested that this could indicate the involvement of hormonal factors.(14)
Another medication implicated in VVS is fluorouracil, an anti-cancer drug that is also being used to treat stubborn genital warts. The Goetch study noted that the two worst cases were seen in women taking this drug.
Systemic corticosteroids, given for serious autoimmune diseases, and topical corticosteroids (often used to treat persistent vulvar itching) are also a possible cause of vulvar pain. These are also treatments for allergic reactions: however, one study suggests that allergy or localized immune reaction is NOT a common cause of vestibulitis.(33) Topical corticosteroid creams in particular may cause thinning and sloughing off of the top layers of skin where they are applied. Both systemic and topical corticosteroids can also cause worsening of undiagnosed infections by counteracting the immune response that causes inflammation, but that also fights the infection as well. There are also anecdotal reports that some women develop VVS after taking Accutane. This needs to be further studied.
The Fibromyalgia Network mentions that vulvodynia is often found in women with Fibromyalgia Syndrome (FMS), which is a muscular pain disorder of uncertain etiology, and which is related to Chronic Fatigue Syndrome. Abnormally high levels of a neurotransmitter involved in regulating pain sensation, called Substance P, have been found in people with FMS and it's possible that at least some cases of VVS are due to abnormalities of the pain perception mechanisms in the body. (15) It's interesting that genetic factors in VVS were suggested by Goetch, (7) because FMS is believed by many researchers to also involve genetic predisposition.
There is also the possibility that abnormally high levels of urine oxalate may be involved. Drs. Clive Solomons, M.H. Melmed, and Susan Heitler of Rose Medical Center in Denver, Colorado have suggested that oxalate may be irritating the vulvar tissues during urination, and are currently conducting a study to see if neutralizing oxalates by taking oral doses of calcium citrate is of value to treat VVS. (16)
A recent study suggests that, while oxalates may be an aggravating factor for some women with vulvodynia, it does not appear to be causative of the condition. Drs. Baggish, Sze and Johnson of the Department of OB-GYN at Good Samaritan Hospital in Cincinnati, Ohio conducted a controlled study comparing urinary oxalate levels in women with vulvodynia and asymptomatic healthy controls. 130 patients with vulvodynia and 23 control volunteers had their 24 hour urinary excretion of oxalate measured. In addition to measuring 24 hour volume and concentration, peak oxalate levels were measured by the hour. There was no difference between the vulvodynia and control groups in any of the measured oxalate parameters, although women with vulvodynia had significantly more frequent voidings. Of this group, 59 patients were treated with a low oxalate diet and calcium citrate for 3 months and then evaluated for response. The study found that 24% of the oxalate treatment group (14 women) had an objective improvement but only 10% (6 women) could have sexual intercourse without pain. (39) The low treatment response rate is discouraging; however, some women may still benefit from the anti-oxalate treatment.
One study mentions alterations in vaginal pH as another common finding.(11)
Finally, a number of immune changes have been found in vulvodynia, although their significance remains unclear. While a detailed discussion of immunology is outside the scope of this paper, the findings are presented below:
The Wayne State University researchers mentioned previously also found mast cells in the patient group. Mast cells are associated with inflammatory allergic reactions and are also found in interstitial cystitis.(42)
In a study at Johns Hopkins in Baltimore, researchers looked for levels of two cytokines (immune stimulating chemicals) associated with inflammation: interleukin-1 beta and tumor necrosis factor-alpha. They found significantly higher levels of both these chemicals in women with VVS when compared to women without vulvar pain. One especially interesting aspect of their study is that the levels of these cytokines was *lowest* closest to the site of the greatest pain.(43)
Pathologists at University Hospital Dijkzigt in Rotterdam, in the Netherlands, did a histopathologic study of biopsies from 12 patients with VVS and 12 age matched controls. They found a chronic inflammatory infiltrate in all of the patients with vestibulitis and none in the control group. This infiltrate was composed of T-lymphocytes, with small numbers of B cells, plasma cells, mast cells and occasional monocytes. An immune globulin important in antibody reactions, IgG, was found in plasma cells of 75% of the test group. This indicates chronic inflammation and possibly an autoimmune origin; however the researchers stated that an autoimmune etiology can neither be confirmed nor rejected based on this study.
They also observed some possibly pre-cancerous changes in the cells of two patients (mild dysplasia), which is interesting in light of the fact that neither the patient nor the control group showed any sign of carcinogenic HPV strains.
To quote their abstract: " Minor vestibular glands were observed in 8 (66%) patients and were associated with a periglandular inflammatory infiltrate. Squamous metaplasia was observed in 4 (44%) patients. Epithelial hyperplasia was present in 10 (83%) patients with mild dysplasia in 2 (16%)." (44)
This might be partly explained by another intriguing immunologic finding. Researchers at the University of Iowa found impaired natural killer lymphocyte activity in VVS when compared to healthy controls. Natural killer cells are a key part of the body's defense system against cancers. Normally, the activity of natural killer cells increases in response to high levels of interleukin-1 and interferon-alpha.(45)
Finally, researchers in Boston found some similarities between VVS and interstitial cystitis when cells obtained by biopsy were stained with a acid-Schiff/colloidal iron stain and with Van Gieson counterstain were compared to biopsies from healthy patients. In interstitial cystitis, cells stained with this process fluoresce. They found immnofluorescence in VVS and suggest that vascular injury associated with altered central neuronal processing could explain the positive immunofluorescence findings in both VVS and IC.(46)
There is no specific test for vulvodynia per se. The diagnosis is based on medical history, looking for redness, swelling and/or pain, and ruling out other illnesses. There are a number of tests that should be done to both rule out other illnesses and to look for infection or another treatable cause of the symptoms.
Many cases are initially diagnosed when women who have pain with intercourse consult a doctor. Other cases are often detected only after many failed attempts, either by the woman herself or by her physician, to treat what appears to be a chronic vaginal bacterial or yeast infection. It's very important to seek a proper diagnosis from a physician or other qualified health provider such as a nurse practitioner or physician's assistant. This is because there are some very serious conditions that can cause similar symptoms.
Among the conditions that must be distinguished from vestibulitis are:
First, a physician or other practitioner should do a careful visual inspection of the area, looking for obvious ulcerations, genital warts, herpes sores, inflammation of the Bartholin's glands (at the base of the vaginal opening) or inflammation of the Skene's ducts on the external vulva. He or she should take vaginal slides and cultures to rule out common and uncommon vaginal infections. In addition to slides for trichomonas, bacteria and yeast, it is important to rule out sexually transmitted diseases such as chlamydia and gonorrhea. Any unusual secretion from the urethra, the Bartholin's glands or the Skene's glands should be cultured, and a pap smear should be taken if any genital warts are noted. It is also necessary to culture for unusual organisms such as ureaplasma or mycoplasma because these organisms often don't show up on a routine culture. In persons with urinary symptoms, urine and urethral cultures should be taken. Urethral cultures are particularly important: sometimes a urethral infection will not show up on a urine culture and can greatly contribute towards the woman's pain. One physician also recommends testing the vaginal pH, since too much acidity in the vaginal secretions may contribute to vulvar inflammation.
The practitioner should take a cotton-tipped swab and gently touch various areas of the vulva to see if the pain can be localized to one area. Often, women who have previously described the pain as around the inner vaginal lips will find that the pain is actually in the hymen itself when the swab is used.
This is often a good time to educate women about vulvar anatomy. Many women believe that the hymen disappears after first sexual intercourse. Actually, this is not true: the hymen remains but is torn. A woman can locate her hymen by taking a mirror and flashlight and inspecting the opening to the vagina. A woman who has had sexual intercourse before will probably notice several "petals", often described as flower-like, directly at the opening to the vagina. A woman or girl who has never experienced vaginal penetration will usually find a kind of membrane or ring around the entrance to the vagina that considerably narrows the vaginal opening.
The practitioner should wipe the vulva with a mild solution of acetic acid (read here: vinegar and water) and then view it under a blue light to see if any areas turn white. These may be areas which have been infected with the Human Papilloma Virus (HPV) - although this is disputed; one paper suggests that acetic acid does not provide a very good guideline as to HPV infections. (32) It may be necessary to examine these areas with a culposcope (a special kind of microscope that lets the practitioner view the cells while still in place) to check for both signs of the virus and for cancer, since HPV is implicated in both cervical and vulvar cancers. Depending on what is seen, a biopsy may be needed. Biopsies in VVS often reveal inflammatory cells; however, at least one paper suggests that inflammatory cells can be seen in women who have no vulvar symptoms at all and thus may be a normal finding.
People researching the connection between vulvodynia and interstitial cystitis have recently developed an interesting test. One theory about painful urogenital syndromes is that the cell walls lining the mucosa of the area have become "leaky" and have altered permeability to potassium ions. One study compared the infusion of small amounts of saline solution with potassium chloride of the same concentration in patients with interstitial cystitis, benign prostatic hypertrophy (BPH), muscular contraction disorders (detrusor instability), acute urinary tract infection and healthy controls. These researchers infused about 40-50 mL of one solution into the bladder, removed it and then infused the same amount of the other solution and tried to see if the patient could tell the difference.
To quote their results: " Neither normal subjects nor patients with interstitial cystitis reacted to water
administered intravesically. There was marked sensitivity to intravesical potassium in 75% of patients with interstitial
cystitis versus 4% of controls (p <0.01). Only 1 patient with BPH responded to potassium and none of the 5 with
chronic urinary tract infection responded. All 4 patients (100%) with a current acute urinary tract infection reacted
positively to the potassium challenge. Of 16 patients with detrusor instability 25% responded." Thus, this
test may prove to be helpful in determining if a particular case of vulvodynia is inflammatory or neurologic. It
may be of particular help to vulvodynia patients with urinary symptoms.(41)
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