3-Bornstein - The consensus.

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Review

The consensus terminology of persistent vulvar pain and

vulvodynia
JACOB BORNSTEIN
Department of Obstetrics & Gynecology, Galilee Medical Center - Nahariya, Israel

INTRODuCTION DEfINITION Of vulvODyNIA

vulvar pain is an enigma. Its etiology, pathophysiology The new definition of vulvodynia is “vulvar pain of at
and treatment have not yet been elucidated. This condition least three months’ duration, without clear identifiable
is presented with pain during intercourse. cause, which may have potential associated factors”.
Around the enigma of vulvar pain, myths about its causes
and treatments that have not been proved to be effective
emerged. However, in 2015, an evidence based consensus DESCRIpTORS Of vulvODyNIA
terminology has been introduced (“2015 terminology”) 1,2,3,
The 2015 terminology further characterizes vulvodynia
with a clear definition and understanding of vulvar pain.
based on location (vestibulodynia, Cliterodynia, general-
Consequently, a new paradigm to the treatment of vulvar
ized, mixed), provocation (upon contact or spontaneous),
pain was developed making it etiology-based.
temporal pattern (intermittent or constant), and onset (pri-
mary or secondary).
Of the various descriptors that are included in the 2015
“vulvAR pAIN”, “vulvODyNIA” OR terminology, the most important is its localization (local-
“DySpAREuNIA”? ized or generalized) and relation to provocation (provoked
for years, the International Society of vulvovaginal and spontaneous) of vulvodynia. Generalized vulvodynia
Disease (ISSvD) discussed “vulvar pain”, but this is some- (formerly termed ‘essential’ or ‘dysesthetic’ vulvodynia af-
what misleading. In fact, the most common and disturbing fects the whole vulva and is usually spontaneous. It is re-
presenting symptom is introital pain during intercourse, i.e. garded as a neuropathic pain and affects postmenopausal
superficial dyspareunia. Dyspareunia is one of the most women mainly.
common complaints associated with sexual dysfunction. In addition, the onset of vulvodynia significantly matters
why has the ISSvD chosen to focus on “vulvar pain” and to treatment outcome4. lpv that has been present since the
not on “Dyspareunia”? There are several reasons to this. first attempt of vaginal penetration is termed primary. If
One is an attempt of the ISSvD to broaden the terminology lpv started after a period of pain-free intercourse it is
so that it involves pain in general and not only pain during named “secondary” lpv. Several researchers believe that
intercourse. An additional reason may be to move away primary lpv is difficult to treat than secondary.
from the limited psycho-sexual connotation of dyspareunia The severity of lpv is determined by the patient’s level
that prevailed years ago. of pain during vaginal intercourse (dyspareunia), using the
Marinoff criteria5: level 1 – dyspareunia causes discomfort
but does not prevent sexual intercourse; level 2: dyspareu-
nia sometimes prevents sexual intercourse; level 3: dys-
THE 2015 TERMINOlOGy
pareunia completely prevents sexual intercourse. However,
The 2015 consensus terminology of “persistent vulvar when intercourse is not practiced, a tampon insertion may
pain and vulvodynia” (table 1) has been created by three in- be used to determine severity of lpv and evaluate the suc-
ternational societies: the ISSvD, the International Society cess of therapy.6
for The Study of women’s Sexual Health (ISSwSH) and The significance of determining severity of lpv is that
the International pelvic pain Society (IppS). The new ter- the approach to treatment should be determined according
minology was achieved in four steps. The first involved a to the severity of the condition, e.g. In level 1 cases, treat-
terminology consensus conference with representatives of ment should not involve surgery. In many cases there is de-
the three societies, held in April 2015. Then, an analysis of terioration of lpv severity with time, and lpv that was
the relevant published studies was used to establish a level level 1 may become level 3. less frequently, a level 3 lpv
of evidence for each factor associated with vulvodynia. The will spontaneously resolve or become a level 1 in severity.
terminology was amended based on feedback from mem- In other cases, treatment will reduce the level of sensitivity,
bers of the societies. finally, each society’s board accepted rather than leading to a complete resolution of the pain.
the new terminology.
The final terminology was simultaneously published by
three journals1,2,3. fACTORS ASSOCIATED wITH vulvODyNIA
The most important innovation of the 2015 terminology
is an appendix table (Table 2) with a list of potential asso-
CATEGORIES Of pERSISTENT vulvAR pAIN
ciated factors (musculoskeletal, neuroproliferation, associ-
The 2015 terminology divides ‘persistent vulvar pain’ in- ated co-morbidities, psychosocial factors, etc) acknowledg-
to two categories: vulvar pain that its cause is known, so ing that vulvodynia likely is not one disease, but several
that it is related to a specific disorder (e.g., inflammatory, disease processes. Only few recognize the significance of
neoplastic, traumatic, infection-related, neurologic, trau- that “appendix” to the consensus terminology, but these
matic, iatrogenic, and hormonal) and ‘vulvodynia’. “potential associated factors” are helpful in identifying pos-

pelviperineology 2018; 37: 3-5 http://www.pelviperineology.org 3

3-Bornstein - The consensus.qxp_treatment 05/02/18 14:19 Pagina 4

Jacob Bornstein

sible etiologies of vulvodynia. So far, no etiology of vulvo- Genetic predisposition of vulvodynia

dynia has been recognized by the ISSvD. Hence, the new Genetic studies have focused on two mechanisms:
terminology revolutionized the approach to the study and – An inability to end a local incident of infection or inflam-
management of vulvodynia, which now need to be individ- mation;
ualized, according to the associated factor. The data on – An increased susceptibility to hormonal changes caused
each associated factor is detailed in a recent review7, and by oral contraceptive pills.
presented below: women with lpv were likely to have the less effective
polymorphism of Mannose-binding lectin (MBl). MBl is
major component of antimicrobial innate immunity, thus
Neuroproliferation or hyperinnervation
An increase in the density of nerve endings in the leading to an increased rate of infections.
vestibular endoderm of women with lpv as compared to furthermore, a loss-of-function mutation in the
controls has been repeatedly documented. These nerve end- melanocortin-1 receptor (MC1R)—which carries anti-in-
ings have been shown to be nociceptors and have an in- flammatory effects, in women with lpv. Addition risk may
creased density of the vanilloid receptor vR1. we have be caused by a loss-of-function mutation in the MC1R gene
shown that the increased density of nerve fibers in women with a variant allele of the Il-1B receptor antagonist gene.
with lpv was 10 times greater than in non-affected wom-
en, and was associated with significant increase in the num-
ber of mast cells and degranulated mast cells within the Musculoskeletal factors
vestibular mucosa. we then demonstrated an increased The association between the pain associated with lpv
subepithelial heparanase activity (degranulated from the and the pelvic floor muscle overactivity may work both
aforementioned mast cells) in the vestibular mucosa. we ways. The dyspareunia frequently results in reflex pelvic
further postulated that histamine, leukotrienes, and nerve muscles contractions and subsequently a permanent in-
growth factor – which are released from the degranulated creased muscle tone. On the other hand, , increased muscle
mast cell – can cause nociceptor proliferation and sensitiza- tension may press on fibers of the pudendal nerve and
tion. In addition, the heparanase, which can degrade the pelvic trauma may lead to nerve damage and myofascial
vestibular stroma, allows these activated and proliferating trigger points. furthermore, it was hypothesized that my-
nociceptors to penetrate through the degraded basement ofascial tissues reflexes activate nociceptive and visceral
membrane into the superficial mucosal epithelium of the neurons.
vestibule. It has been theorized by many groups that certain
genetic polymorphisms may predispose affected women
with lpv to have an exaggerated inflammatory response or Hormonal factors
chronic infection, which leads to mast cell activation and A controversy exists as to whether combined oral hor-
subsequent nociceptor proliferation. monal contraceptives pills (HCp) play a role in the devel-
opment of lpv. Against that association is that of the mil-
lions of women taking HCp, only a slight fraction suffers
Central nervous system involvement in vulvodynia
Central nervous system alterations as a cause of vulvody- from lpv.
nia has been suspected. Several mechanisms have been pro- Indeed, three studies have failed to show an association
posed: between HCps and vulvodynia. A case control study even
• Altered central nervous system processing; showed that HCps actually decreased the risk of vestibulo-
• Activation of the hypothalamic pituitary adrenal (HpA) dynia.
axis via chronic stress; On the other hand, some women with lpv describe an
• visceromotor responses to vaginal distension; improvement with cessation of the HCp, and other studies
• Global sensitization of nociceptive transmission. depicted association of vestibulodynia with HCp and iden-
tified a polymorphism in the androgen receptor that signif-
icantly increased the risk of developing HCp-induced lpv
TABlE 1. 2015 Consensus terminology and classification of persis- in affected women.
tent vulvar pain and vulvodynia with prolonged use of HCp, the net effect is progesto-
genic, so that the estrogen influence on the vestibule and
• Infectious (e.g. recurrent candidiasis, herpes) vagina is reduced. This leads to diminished lubrication and
decreased elasticity, causing increased friability and epithe-
A. Vulvar pain caused by a specific disorder*

• Inflammatory (e.g. lichen sclerosus, lichen planus, immunobul-

lous disorders) lial damage with vaginal intercourse. later, allodynia and
• Neoplastic (e.g. paget disease, squamous cell carcinoma) burning may occur.
• Neurologic (e.g. post-herpetic neuralgia, nerve compression or in-
jury, neuroma)
• Trauma (e.g. female genital cutting, obstetrical) Embryological/Congenital Factors
• Iatrogenic (e.g. post-operative, chemotherapy, radiation)
• Hormonal deficiencies (e.g. genito-urinary syndrome of meno- vulvodynia is sometimes associated with painful bladder
pause [vulvo-vaginal atrophy], lactational amenorrhea) syndrome (interstitial cystitis) and with periumbilical hy-
B. Vulvodynia – vulvar pain of at least 3 months’ duration, without persensitivity. This association dates back to the embryo
clear identifiable cause, which may have potential associated factors development period; the vestibule develops from the uro-
genital sinus, which is contiguous with the allantois, which
• localized (e.g. vestibulodynia, clitorodynia) or Generalized or later differentiates to the urachus and the umbilicus.
Descriptors:

Mixed (localized and generalized)

• provoked (e.g. insertional, contact) or Spontaneous or Mixed
(provoked and spontaneous)
• Onset (primary or secondary) Inflammatory factors
• Temporal pattern (intermittent, persistent, constant, immediate, women with vulvodynia are more likely to have a history
delayed) of allergic rashes. This may clarify the excess of mast cells,
* women may have both a specific disorder (e.g. lichen sclerosus) and vulvody- found in cases with lpv compared to controls. Mast cell
nia. produced tumor necrosis factor (TNf) which has been as-

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The consensus terminology of persistent vulvar pain and vulvodynia

TABlE 2. 2015 Consensus terminology and classification of persis- REfERENCES

tent vulvar pain and vulvodynia
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C, Zolnoun D, Coady D; 2015 ISSvD, ISSwSH, and IppS
• Co-morbidities and other pain syndromes (e.g. painful bladder Consensus Terminology and Classification of persistent
vulvar pain and vulvodynia. consensus vulvar pain terminol-
Appendix: Potential factors associated with Vulvodynia*

syndrome, fibromyalgia, irritable bowel syndrome, temporoman-

dibular disorder) [level of evidence 2] ogy committee of the International Society for the Study of
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• Structural defects (e.g. perineal descent) [level of evidence 3] International pelvic pain Society (IppS). J Sex Med. 2016, 13
* The factors are ranked by alphabetical order.
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C, Zolnoun D, Coady D; 2015 ISSvD, ISSwSH, and IppS
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CONCluSION
The terminology of vulvar pain and vulvodynia was pre-
pared by the ISSvD, ISSwSH, and IppS. It acknowledges
Correspondence to:
professor Jacob Bornstein MD, MpA
the complexity of the clinical presentation and pathophysi- Chairman, Department of Obstetrics & Gynecology,
ology involved in vulvar pain and vulvodynia, and incorpo- Galilee Medical Center - Nahariya, Israel.
rates data from evidence-based studies conducted during Associate Dean, Bar-Ilan university faculty of Medicine
the last decade. The inclusion of factors associated with Tel: +972-4-9107720, fax: +972-4-9001890.
vulvodynia enables novel approach to research, diagnosis Mobile: +972-50-7887631.
and treatment. e-mail: [email protected]