Alessandra Graziottin1, 2 and Filippo Murina3
(1)
Center of Gynecology and Medical Sexology, H. San Raffaele Resnati, Milan, Italy
(2)
Graziottin Foundation for the cure and care of pain in women, Italy
(3)
Department of Vulvar Disease V. Buzzi Hospital Obstetrics and Gynecological Clinic, University of Milan, Milan, Italy
Abstract
A patient’s medical history provides essential information in the determination of a correct diagnosis of vulvodynia. It is important to ask specific questions in order to obtain detailed information, and equally essential to ask open-ended questions that allow a patient to describe her condition, throughout the whole process, displaying empathy, understanding, and acceptance (see Chapter 3).
Vulvodynia is a diagnosis of exclusion, a pain syndrome with no other identified cause.
Medical History
A patient’s medical history provides essential information in the determination of a correct diagnosis of vulvodynia. It is important to ask specific questions in order to obtain detailed information, and equally essential to ask open-ended questions that allow a patient to describe her condition, throughout the whole process, displaying empathy, understanding, and acceptance (see Chapter 3).
Box 1
The targeted history of vulvodynia patients
|
• Current symptoms |
|
• Personal habits (hygiene, typical diet, etc.) |
|
• Past medical and surgical history |
|
• Obstetric and gynecologic history (menstrual function, contraception, previous vulvar infections, etc.) |
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• Medications and allergies |
|
• Sexual history and psychological profile |
|
• Bladder and bowel function |
Personal habits is a fundamental aspect to consider; eliminating all possible irritants is an important step. If patients are using topical creams with an irritating base - as is often the case - they have to inform the clinician. The daily use of a potentially irritating mini-pad or panty-liner is not healthy for any woman and many lubricants also contain irritants as preservatives.
It is also important to recognize certain aspects of a patient’s medical history such as bladder and bowel function. It has been estimated that more than half of vulvodynia patients have symptoms of excessive urgency and frequency of urination and suprapubic pain. This condition is defined painful bladder syndrome/interstitial cystitis (PBS/IC). PBS/IC is a chronic, severely debilitating disease of the urinary bladder with a course that is usually marked by flare-ups and remissions. Dyspareunia is not uncommon in persons with PBS/IC and may be related to the mechanical effects of intercourse on the inflamed bladder.
Box 2
Diagnostic criteria for painful bladder syndrome /interstitial cystitis (PBS/IC)
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• Presence of urinary urgency or frequency, or pelvic/perineal or bladder pain |
|
• Presence of glomerulations (pinpoint submucosal hemorrhages) or ulcers on cystoscopic examination with hydrodistension under anesthesia (interstitial cystitis) |
|
• Absence of genitourinary infections |
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• Absence of neoplastic diseases or benign bladder tumor |
|
• Absence of history of radiation, tuberculosis or chemical cystitis |
Irritable bowel syndrome (IBS) is a very common functional gastrointestinal disorder characterized by abdominal discomfort, bloating, and disturbed defecation. Many IBS patients have at least one comorbid somatic complaint and many meet diagnostic criteria for other functional disorders
Several studies have reported an increased prevalence of sexual dysfunction among IBS patients, including increased dyspareunia and more severe IBS symptoms following intercourse. The studies are consistent in their finding that the overlap among these disorders (vulvodynia - PBS/IC - IBS) is greater than expected based on their separate prevalence rates. In addition, patients with more than one disorder have greater disease severity, higher rates of psychopathology, and more severely impaired healthrelated quality of life than those with only one disorder.
It is also essential to develop a history of medication use and compare it to the timeline of the patient’s vulvodynia history. Many vulvodynia patients use prescription medications for anxiety and depression, often secondary to vulvar pain. Sexual dysfunction may affect the majority of patients taking some psychotropic drugs, as a predictable outcome of the same neurotransmitter effects that underlie the agents’ therapeutic action. Such predictable forms of sexual dysfunction include the orgasmic disturbances and the diminishment of sexual desire associated with serotonin-reuptake inhibitor antidepressants (SSRIs) and serotonin-norepinephrine reuptake inhibitors (SNRIs), such as venlafaxine. Benzodiazepines, particularly in the higher doses often used for panic disorder, have been most strongly associated with decreased libido.
Antibiotics are a frequent prescription medication that vulvodynia patients use as a consequence of a vaginal swab; in fact we can find commensal bacteria such as streptococci, diphtheroids and coagulase-negative staphylococci without any responsibility on vulvar pain. Antibiotics do not directly cause vulvodynia but their repetitive and improper use are thought to predispose women to vulvovaginal candidosis by eliminating the protective bacterial flora, thus allowing Candida overgrowth in the gastrointestinal tract, vagina, or both. In particular, Lactobacillus spp. could provide colonization resistance and prevent germination, maintaining low numbers of yeast.
The psychosocial evaluation should include information about the presence of psychologic symptoms (e.g., anxiety, depression, anger), psychiatric disorders, personality traits or states, and coping mechanisms. Evidence of stress levels, degree of support from others, including partner or family, should also be noted. It is important to ask women about all aspects of their sexual function and satisfaction (see Chapter 3).
Physical Examination
The first important aspect of physical examination is the visual inspection of vulvar region (Fig. 7.1). It requires a meticulous and methodical examination of the vulva, including the perineum and perianal region.
Box 3
The targeted physical examination of vulvodynia patients
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• Vulvar region: |
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- visual inspection and vulvoscopy |
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- palpation |
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- Q-tip test |
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• Pelvic floor evaluation |
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• Vaginal inspection |
|
• Bi-manual palpation |
Fig. 7.1
Vulvar anatomy
Vulvoscopy, the colposcopic examination of the vulva, gives the examiner the ability to characterize any lesions with much greater detail. For example, the vulvoscopist can determine if a lesion is a raised plaque or just a flat macule. In addition, the margins of the lesion can be characterized (e.g., are the lesions distinct or are the observed changes more diffuse?).
Vulvoscopy enhances the ability to detect:
· Color changes associated with inflammatory or neoplastic diseases of the vulva:
· red areas can be visualized when there are stromal changes due to inflammation, vulvar dermatoses, or neovascularization in association with neoplasia;
· white areas can be found when there is a decreased vascularization, fibrotic changes in the stroma and increased keratinization (lichenification).
· Scarring and architectural changes: Chronic inflammatory disorders of the vulva, such as lichen sclerosus and lichen planus, frequently cause structural changes such as resorption of the labia minora, vulvar granuloma fissuratorum (chronic tearing of the posterior fourchette), and phimosis of the clitoris.
· Subtle lesions such as tiny fissures, which may be very painful.
Therefore, the vulvoscopist’s main goal in suspected vulvodynia is to rule out specific diseases that can cause vulvar pain.
Box 4
Evaluating abnormalities of the vulvar skin
|
Color |
|
Texture |
|
Integrity: flattening of the labia minora, asymmetry, agglutination, synechial formation anteriorly and/or posteriorly |
Diagnoses to Rule out
Skin diseases of the vulva or vagina can also cause pain. Lichen sclerosus, lichen planus, and lichen simplex chronicus are three of the most common non-neoplastic epithelial disorders of the vulva.
Lichen sclerosus (LS) is a non-neoplastic chronic lymphocyte-mediated inflammatory dermatosis with distinctive dermal sclerosis and a predilection for the anogenital skin in women. Itch is the main symptom but pain occurs if there are erosions or fissures. Dyspareunia occurs in the presence of erosions, fissures or introital stenosis. There is typically no genital mucosal involvement, but the stenosis that may develop at the edge of mucocutaneous junctions can cause severe dyspareunia.
The clinical features of lesions are variable depending on the stage and severity of the disease. Patchy involvement is seen in some, while others have extensive, confluent disease (Fig. 7.2). Areas of pale, thinned, wrinkled, atrophic skin, possible telangiectasia and haemorrhagic blisters, may be evident at sites of lesions (Fig. 7.3). Lichenification or hyperkeratosis may also be the prevalent pattern.
Fig. 7.2
Lichen sclerosus
Fig. 7.3
Lichen sclerosus
Progressive sclerosis can lead to loss of normal genital structures. Labia minora may become fused or resorbed, the clitoris may be buried and the introitus significantly narrowed.
LS in females has two peak ages of presentation. The first of these occurs in prepubertal girls (7.4) and may resolve or continue beyond the menarche. The other peak of incidence is in postmenopausal women.
It is important not to confuse childhood sexual or physical abuse with prepubertal lichen sclerosus (Figs. 7.4 and 7.5).
Fig. 7.4
Prepubertal lichen sclerosus
Fig. 7.5
Childhood sexual abuse
Lichen planus (LP) is an inflammatory autoimmune disorder involving keratinized and mucosal surfaces. There are three clinical variants that affect the vulva: erosive lichen planus, papulosquamous lichen planus, and hypertrophic lichen planus.
Vulvovaginal involvement can be associated with itching, burning, pain, dyspareunia, and destruction of the vulvar and vaginal architecture. The variant that typically affects the vulva and vagina is called erosive LP and this is the most painful form of the disease (Fig. 7.6).
Fig. 7.6
Erosive lichen planus
The main symptoms are vulvar burning, occurring spontaneously or after vulvar contact, and severe dyspareunia. In LP the mucosa of the introitus is often denuded with a red, glazed appearance; there may be erythema of the vestibular mucosa with varying degrees of epithelial desquamation or frank erosions.
Lichen simplex chronicus is not a specific entity, but rather describes lichenification of the vulva caused by persistent itching and scratching. The skin can become leathery and thickened or, in severe cases, may be excoriated (Fig. 7.7). Vulvar pain, if present, is usually a result of irritation from open lesions.
Fig. 7.7
Vulvar lichen simplex
Many different diseases may produce erosive, ulcerative, or desquamative lesions of the vulva; vulvar aphthae and genital herpes are the two common ones. Vulvar aphthae are small, shallow ulcers with a yellow base and erythematous rim. They occur acutely and resolve over a few day, and are quite painful. Although attacks tend to be intermittent, they can be very frequent or almost continuous in some patients. Women with vulvar aphthosis frequently have oral aphthae (Fig. 7.8).
Fig. 7.8
Vulvar aphthous ulcer
Vulvar aphthae are commonly confused with genital herpes, and this is hardly surprising, given that they are painful, acute, recurrent ulcers. The difference is that genital herpes, once past the very brief blister stage, has the appearance of an erosion rather than the typical deeper, ulcerative lesions that are seen in the mouth (Fig. 7.9).
Fig. 7.9
Vulvar herpes simplex
The mucosa of the labia minora and vestibule is generally pink and smooth; however, localized or widespread micropapillary or villiform patterns may sometimes be observed (Fig. 7.10). These can be misinterpreted as condyloma due to human papillomavirus (HPV) infection.
Fig. 7.10
Vulvar micropapillomatosis
Vulvar micropapillomatosis should be left untreated; laser removal, for example, frequently results in an ‘iatrogenic’ trigger for vestibulodynia development (Table 7.1).
Table 7.1
Differentiating vulvar micro-papillomatosis from vulvar condilomas
|
Vulvar micropapillomatosis |
Vulvar condilomas |
|
• Regular shape and distribution |
• Small bumps |
|
• Uniform color |
• Flat or verrucous |
|
• Soft consistency |
• Reddish or brown, smooth |
|
• Lack of tendency to fuse |
• Dome-shaped lesion on keratinized skin |
Vulvar intraepithelial neoplasia (VIN) does not have a characteristic presentation. Some patients may have pruritus or burning, while others will notice an asymptomatic abnormality on the vulvar skin. The lesions may be raised or flat with a rough surface. The lesions may appear white or red or of mixed color (Fig. 7.11).
Fig. 7.11
Vulvar intraepithelial neoplasia
Vestibular tenderness is assessed by applying a cotton tipped swab (the Q-tip test; Fig. 7.12) to the vulvar vestibule in a clock-like pattern. Gentle touch provokes either hyperesthesia, a heightened intensity relative the degree of applied pressure, or allodynia, the perception of a different sensation to that applied (e.g., pain rather touch).
Fig. 7.12
Cotton swab test
Thresholds to pain provoked by pressure are markedly lower in provoked vestibulodynia patients. Pain is typically most severe in the posterior vestibule between the 5 and 7 o’clock positions, but can occur anywhere within the vestibule.
The Q-tip touch test has been validated as useful in identifying the exact location of the pain and enabling the patient to classify the areas where it is mild, moderate, or severe. A diagram of pain locations is helpful in assisting the assessment of pain over time.
Provoked vestibulodynia patients have reported significantly higher pain ratings at the vulvar vestibule than pain-free controls, demonstrating the utility of this test in distinguishing between vulvodynia patients and control women.
Pelvic Floor Evaluation
Box 5
Pelvic floor markers to investigate in vulvodynia patients
|
• Tenderness |
|
• Trigger points |
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• Hypertonus |
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• Decreased mobility |
Clinical evaluation of the pelvic floor begins with simple observation of pelvic floor muscle activity during the process of squeezing and relaxation. The simple observation of the perineum and introital area in the dorsal lithotomy position during the performance of a Kegel squeeze is often quite revealing. The woman squeezes the muscles used to stop the flow of urine for about 10 seconds, and then relaxes them for about 10 seconds.
Patients with pelvic floor hypertonic dysfunction often have so much muscle tension at ‘rest’ that they are unable to produce more contractile strength and therefore cannot produce an effective squeeze. It can be revealed by a ‘short perineum’, conventionally with a length above 2 centimeters.
At this point the examiner, if accepted by the patient, should place a generously lubricated single finger in the vagina to assess pelvic floor awareness and the ability to squeeze and relax the levator ani. Many scales are available to document strength, tone, and tenderness, yet all these scales are subjective and unvalidated. We usually used a simple empiric score that allows us to reproduce pelvic floor hypertonus with an acceptable reliability.
Box 6
Pelvic floor hypertonicity score
|
0 No hypertonicity |
|
1 Mild hypertonicity |
|
2 Moderate hypertonicity |
|
3 Severe hypertonicity |
Many patients will be found to be most tender along the lateral border of the levator ani, which is where the levator muscles insert onto the arcus tendineus levator ani.
Muscular pain can be assessed with insertion of one finger at the introitus as the patient performs a series of contraction and relaxation exercises. Spontaneous or elicited pain in the lower third of the anterior vaginal wall should be carefully explored, as it may be associated with bladder-related comorbidities (cystalgia, urethralgia, post-coital cystitis, intersitial cystitis) that are reported in one third of vulvodynia patients.
Vaginal Inspection
The vagina should be examined for possible evidence of atrophy, ulcerations or abnormal discharge. Secretions should be collected from the lateral vaginal walls using a swab. It is recommended that in the assessment of women with vaginal discharge (Table 7.2), vaginal pH is checked, using narrow range paper (pH 4–7). pH is normal (4.0–4.5) in vulvovaginal candidosis, and pH in excess of 4.7 usually indicates bacterial vaginosis, trichomoniasis, or a mixed infection. A wet mount or saline preparation should be done routinely to identify the presence of yeast cells and mycelia but also to exclude the presence of so-called clue cells indicative of bacterial vaginosis and motile trichomonads. A 10% potassium hydroxide preparation is more sensitive than a saline preparation in identifying yeast or hyphae (65–85% sensitivity).
Table 7.2
Causes of vaginal discharge in women of reproductive age
|
Physiological |
|
Infective (non-sexually transmitted) |
|
- bacterial vaginosis |
|
- Candida |
|
Infective (sexually transmitted) |
|
- Trichomonas vaginalis |
|
- Chlamydia tachomatis |
|
- Neisseria gonorrhoeae |
|
Non-infective |
|
- foreign bodies (tampons, condom) |
|
- cervical polyps or ectopy |
|
- genital tract malignancies |
Tests
Histopathology, traditionally, has been of little value except to exclude other conditions. Biopsies are not generally performed when the physical examination and history have ruled everything else out. The routine use of magnetic resonance imaging (MRI) is usually recommended in patients with unprovoked pain. MRI may not be necessary, however, as the incidence of pathology, for example sacral cysts, causing referred pain to the vulva is very low. Pelvic floor surface electromyography (EMG) is a test that should not be performed routinely.
Objective identification of pelvic floor hypertonic dysfunction can be obtained using various techniques. The most common is surface EMG, which is often performed as a part of a pelvic floor evaluation by physical therapists and nurse clinicians trained in the evaluation and management of patients with pelvic floor dysfunction. In patients with hypertonic dysfunction we find the following (listed in order of prevalence):
· elevated and unstable resting baseline activity;
· poor recovery, poor postcontraction and relaxation;
· spasms with sustained contractions and poor strength.