Clinical Management of Vulvodynia: Tips and Tricks 2011th Ed.

2. Vulvodynia - What’s Behind the Name?

Alessandra Graziottin^{1, 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

Vulvodynia includes and encompasses a number of heterogeneous vulvar conditions, with different etiologies and pathophysiologies, and a common symptom: chronic vulvar pain.

‘Vulvodynia’ is a diagnostic term referring to chronic pain in the vulvar area of at least 3 months duration.

Vulvodynia includes and encompasses a number of heterogeneous vulvar conditions, with different etiologies and pathophysiologies, and a common symptom: chronic vulvar pain.

Characteristics and Etiology of Vulvar Pain

Vulvodynia may be chronic or unremitting, intermittent or episodic (often exacerbated premenstrually). It may be unprovoked (spontaneous) or it may occur only in response to a touch stimulus (provoked pain), including tight clothing or physical stimulation in the vulvar area (such as with coitus or pelvic examination). It may be generalized, involving all the vulvar area, or localized, limited to the vestibular area (defined as ‘vulvar vestibulitis’, or VV), to the clitoris (‘clitoralgia’), to the peri-urethral mucosa and/or to a limited part of the vulva (Fig. 2.1).

Fig. 2.1

Vulvodynia may be generalized, involving all the vulvar area, or localized, limited to the vestibular area (defined as vulvar vestibulitis, or VV), to the clitoris (clitoralgia), to the peri-urethral mucosa, and/or to a limited part of the vulva

It may be useful to clinically describe vulvar pain using a ‘pain map’ that describes the site and the intensity of pain derived from the woman’s history, and the symptoms reported by the woman during vulvar and pelvic examination.

There is no consensus about the use of terms to define and subtype conditions underlying vulvodynia. However, other descriptors may be clinically useful. For example, vulvodynia can be the only complaint (‘isolated’ vulvodynia), or it may be comorbid with other conditions:

1.

Medical: vulvo-vaginal Candida infections, vulvar dystrophies and neoplasms, contact dermatitis, hypoestrogenic and hypoandrogenic atrophy, painful bladder syndrome, endometriosis, irritable bowel syndrome, fibromyalgia, headache. Other recognized medical disorders with a possible chronic vulvar pain component include neurologic etiologies (pudendal nerve entrapment syndrome, multiple sclerosis), referred pain from myalgic muscles (levator ani), iatrogenic conditions (painful outcome after perineal surgery such as episiorraphy, hemorroidectomy, or posterior colpectomy), or pelvic/perineal radiotherapy;

Box 1

Iatrogenic Vulvodynia

Key point: Physicians should increase their attention to the potential iatrogenic role implicit in their diagnostic or therapeutic behaviors involving the pelvic organs, vulva and perineum at any age in the lifespan.

2.

Sexual: coital pain (“dyspareunia”) is the most frequently reported sexual problem alongside loss of desire, vaginal dryness, orgasmic difficulties, especially during intercourse. In severe cases, sexual aversion may be reported.

Table 2.1

The characteristics of vulvodynia

Definitions of Vulvodynia

Definitions of vulvodynia have varied widely, mirroring the difficulties in understanding and substantiating the clinical reality and the pathophysiology of vulvar pain. Of interest and concern is the fact that the condition was not officially recognized until 1898, was overlooked for more than 80 years, and then resurfaced in gynecological texts in the 1980s. The working definition of vulvodynia used by a 2005 consensus panel was ‘chronic pain lasting from three to six months in the vulvar region without another definable cause.’ The most recent terminology and classification of vulvar pain by the International Society for the Study of Vulvovaginal Disease (ISSVD) divides potential causes of vulvar pain into four categories:

·               infectious;

·               inflammatory;

·               neoplastic;

·               neurologic.

Conditions falling into these categories must be ruled out prior to making a diagnosis of vulvodynia, defined as “vulvar discomfort, most often described as burning pain, occurring in the absence of relevant visible findings or a specific, clinically identifiable, neurologic disorder.” Vulvodynia is not caused by infection (candidiasis, herpes, etc.), inflammation (lichen planus, immunobullous disorder, etc.), neoplasia (Paget’s disease, squamous cell carcinoma, etc.), nor is it a neurologic disorder (herpes neuralgia, spinal nerve compression, etc.).

The classification of vulvodynia is based upon the site of the pain, whether it is generalized or localized, and whether it is provoked, unprovoked, or mixed. The ISSVD further classifies vulvodynia as follows:

·               Generalized vulvodynia

·                      provoked (sexual, nonsexual, or both);

·                      unprovoked;

·                      mixed (provoked and unprovoked);

·               Localized vulvodynia

·                      provoked (sexual, nonsexual, or both);

·                              provoked vestibulodynia/Vulvar Vestibulitis Syndrome;

·                      unprovoked;

·                      mixed (provoked and unprovoked).

TIP: Unfortunately, these definitions limit vulvodynia to a subset of unexplained vulvar pain, thus missing those conditions where pain has a clear etiology. Opposite to that, we suggest that vulvodynia should include all types of vulvar pain. It is the physician’s responsibility to make a differential diagnosis among the different biological etiologies of vulvar pain, focusing on pathophysiology and the histology of vulvar tissue. Vulvo dynia can be exacerbated by psychobiological factors (anxiety, depression, chronic stress, former abuse) and sexual triggers such as intercourse.

Indeed, pain (almost) always has a biological etiology (the only exception being pain from grief) and what may not be immediately visible at first vulvar examination may become evident when appropriate and skilled clinical examination is performed and/or when histological data express clear evidence of an inflammatory condition typical, for example, of vulvar vestibulitis.

Impact of Vulvodynia on Physical and Psychosexual Health

Vulvodynia is a prevalent and highly distressing disorder, with major health, psychosexual, interpersonal and social consequences.

·               Health-related issues: Besides being a serious medical problem, vulvodynia may trigger a process whereby the pain spreads to become a real ‘red alert’ in the pelvis. As a chronic inflammatory process, vulvar pain may secondarily involve/extend to other pelvic organs: the most frequent comorbidity being with bladder symptoms (post-coital cystitis, bladder pain syndrome). Other significant associations include endometriosis, chronic pelvic pain, irritable bowel syndrome, fibromyalgia, chronic fatigue syndrome, coccygodynia, headache and anxiety/depression. Preliminary evidence suggests a pathophysiology for comorbidities that involves:

1.

A chronic inflammatory process involving different pelvic organs. The common denominator seems to be the hyperactivity of mast cells (Fig. 2.2), which produce and release molecules that are responsible for the local inflammatory process, for the activation of the pain system, and for the defensive contraction of muscles in the painful area. Mast cells travel throughout the body, specifically patrolling the body’s ‘boundaries’ such as the colonic mucosa, the bladder mucosa and the vestibular area. They are the powerful directors of the inflammatory orchestra, and the mediators of the shift from acute inflammation with nociceptive pain to chronic inflammation with a further step towards neuropathic pain. This may help to explain how comorbidities arise among organs and systems that are located at different sites;

2.

Nerves innervating the organs that are in close proximity to the vulva (for example, the pudendal nerve). The term ‘cross-talk’ has been used to express this process of ‘sharing pain’ between different organs sharing a common innervation. Proliferation of the pain nerve endings in the inflammated tissue, mediated and induced by the Nerve Growth Factor (NGF) produced by the hyperactivated mast cell has been histologically proven.

3.

The hyperactivity - i.e. the excessive contraction - of the levator ani, the key muscle of the pelvic floor. This contraction can be either lifelong (“myogenic”) and associated to early symptoms such as lifelong constipation and/or bladder symptoms such as recurret cystitis from colonic germs urge and/or recurrent vaginitis.

·               Psychosexual issues: having pain in a ‘secret’ area of the body, difficulties in disclosing it to others, and/or being medically labeled as ‘inventing the pain’, may trigger a sense of isolation in women - of being ‘the only one’ with such an embarrassing and disabling condition. This suffering may be worsened if the woman has been harassed or abused in the past: the pain can act as a reminder of what she has suffered; it can be associated with unspecified feelings of guilt or be perceived as ‘retribution’ for perceived inappropriate sexual desire, masturbation, or affairs; and it can be associated with post-traumatic stress disorder. As unwanted pain is the strongest reflex inhibitor of desire, of mental and physical arousal, vulvodynia is associated with the progressive inhibition of the sexual response, with low levels of desire, vaginal dryness, orgasmic (coital) difficulties and increasing dissatisfaction or frank frustration with sexual intimacy. Chronic pain, of whatever type, can destroy a person’s vitality, leaving the patient weak, fatigued, anergic, moody, fearful, distressed, depressed, and pessimistic to the degree of frank catastrophism, a shadow of the person they once were.

·               Interpersonal and social issues:

a)

For the couple: having a partner who complains of chronic genital pain is a challenge even for the most loving of companions, for a number of reasons: 1) it can chronically limit sexual intimacy up to to the point of frank avoidance of any intimate behavior; 2) it can monopolize the conversation and focus the couple on the vulvar pain and its related symptoms; 3) it can irritate and causes anger, aggressiveness, and frank verbal and physical abuse, especially if the woman’s physician tells the partner that: “The pain is all in her head”; that, “She is inventing the pain”; or that, “She is just trying to avoid having intercourse”; 4) it has increasing costs, both monetary (for physician visits, physical examinations, loss of working days) and related to quality of life (the social and emotional impact on a relationship);

b)

In the family: when a mother is unwell, the children may be aware that something is wrong, and may feel deprived of attention and tenderness - a problem that can increase as the condition becomes more severe;

c)

At work: women with vulvodynia report increased loss of working days, as well as increased difficulties in concentrating or even staying seated at their desk. Many women go part time or leave their jobs, and can feel forced out of work.

Fig. 2.2

Mast cells picture, rounded elements with an oval nucleus and cytoplasm with spherical granules containing cytokines, growth factors, vasoactive amines and proteolitic enzymes. Mast cells activated by various stimuli selectively release inflammatory factors that mediate the typical signs and symptoms of local inflammation including erythema, edema, increased local temperature, pain and functional impairment. When persistently up-regulated, mast cells maintain chronic inflammation, leading to a shift between nociceptive and neuropathic pain

Conclusions

Vulvodynia is a highly prevalent and serious condition that deserves to be diagnosed and cured in order to reduce the suffering of both women and their partners; to avoid progressive worsening to chronicity and comorbidities; to reduce personal, family and social costs; and to return women and their partners to a happier personal and intimate life. Vulvodynia is a legitimate medical entity, with a challenging spectrum of etiologies and clinical presentations, that deserves a rigorous, comprehensive and multidisciplinary approach.