VULVAL [VULVAR] PAIN (VULVODYNIA)

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CURRENT ICS DEFINITIONS
Derived from Standardisation of Terminology of LUT function (Abrams P, et al 2002)
- Vulval pain is felt in and around the external genitalia.
- Vaginal pain is felt internally, above the introitus.
Taxonomy: Lower urinary tract symptoms (LUTS): 1.6 Genital and Lower Urinary Tract Pain
- Vulval pain syndrome is the occurrence of persistent or recurrent episodic vulval pain, which is either related to the micturition cycle or associated with symptoms suggestive of urinary tract or sexual dysfunction. There is no proven infection or other obvious pathology[10].
Note 10: The ICS suggests that the term vulvodynia (vulva – pain) should not be used, as it leads to confusion between single symptom and a syndrome.
- Vaginal pain syndrome is the occurrence of persistent or recurrent episodic vaginal pain which is associated with symptoms suggestive of urinary tract or sexual dysfunction. There is no proven vaginal infection or other obvious pathology
Taxonomy: Lower urinary tract symptoms (LUTS): 1.7 Genito-Urinary Pain Syndromes and Symptom Syndromes Suggestive of LUTD
ICD CODING
2012 ICD-9-CM Diagnosis Codes > Diseases Of The Genitourinary System 580-629 > Other Disorders Of Female Genital Tract
International Classification of Diseases: ICD-9, group 625 Pain and other symptoms associated with female genital organs, and specifically ICD-9 625.7
HISTORY
Sources:
- Moyal-Barracco M, Lynch PJ.//2003 ISSVD terminology and classification of vulvodynia: a historical perspective.// J Reprod Med. 2004 Oct;49(10):772-7.
- Haefner, HK. //Report of the International Society for the Study of Vulvovaginal Disease Terminology and Classification of Vulvodynia//. J Low Gen Tract Dis. 2007; 11(1):48-9.
- Veasley C, Marinoff S. The Evolution of Vulvodynia Terminology: Clarifying the Issue, in NVA News, Vol IX, Issue III. Summer 2004, pages 5-7
In 1889, in a treatise on the diseases of women, Alexander J.C. Skene (1837-1900), a Professor in the Diseases of Women from Brooklyn, described the symptoms of vulvar pain, describing a disease “characterized by a supersensitiveness of the vulva… When, however, the examining finger comes in contact with the hyperaesthetic part, the patient complains of pain, which is sometimes so great as to cause her to cry out”.
Skene AJC. Treatise on the Diseases of Women, New York: D. Appleton and co, 1889:93-4
However, little attention was paid to this area of female diseases, explained in 1012 by Howard Atwood Kelly, (1858-1943) an eminent surgeon with a great interest in the genitourinary diseases of women from the John Hopkins University School of Medicine in Baltimore as follows:
“Previous to the latter half of the nineteenth century but little was known about diseases of the urinary apparatus in women. And while the relatively more urgent and dangerous diseases of the male organs had exacted the closest attention, the modesty of women, as well as the inaccessible nature of the affections, conspired to hinder an earlier scientific investigation of their genito-urinary organs.”
Kelly HA. Operative Gynecology. 2nd Edition 1912. D. Appleton and Company
In 1928, Kelly also addressed this specific topic of vulval pain, writing that “exquisitely sensitive deep-red spots in the mucosa of the hymeneal ring are a fruitful source of dyspareunia – tender enough at times to make a vaginal exam impossible.”
Kelly HA, D. New York: Appleton and Co;1928:235-9.
For several decades after this, female afflictions of the genital organs, particularly those affecting sexuality, received scant attention.
1975 a change came about in 1975 at the World Congress of the International Society for the Study of Vulvovaginal Disease (ISSVD) at which a plea was made to members by Dr Esther Weisfogel to investigate what she described as “the burning vulva”. This led to much discussion and adoption by the ISSVD of idiopathic vulvar pain as an entity, termed “burning vulva syndrome”.
1978 Dodson and Friedrich described a condition with vulvovaginal pain and dyspareunia in the absence of identifiable clinical findings. They termed this “psychosomatic vulvovaginitis”, a term which may have done more harm than good for many years to come.
Dodson MG, Friedrich EG Jr. //Psychosomatic vulvovaginitis//. Obstet Gynecol 1978;51(1 Suppl):23s-25s.
The term “vulvodynia” first appeared in a publication also in 1978 by Tovell and Young in a first classification of vulvar diseases based on clinical experience with some 1,000 patients, 26 of whom had “…pruritis, discomfort and pain that was often burning in nature. Neither clinical nor laboratory evidence of a local or systemic disease could be discerned…”
Tovell MM, Young AW Jr. //Classification of vulvar diseases//. Clin Obstet Gynecol 1978 21:955-961.
1983 Friedrich wrote that “vulvar pain, burning and dyspareunia are challenging complaints that often defy attempts at specific diagnosis. The vulvar vestibule is being recognized increasingly as a site of these symptoms, but the entities found at this location are not recognized widely. The clinical and histologic features of the minor vestibular glands, mucinous cysts, vestibular adenitis and vestibular papillae are given as they appeared in 28 patients”.
Friedrich EG Jr. The vulvar vestibule. J Reprod Med 1983 28:773-77.
A Task Force on Vulvar Pain was set up at the 6th ISSVD World Congress in 1981 and the results presented at the following World Congress in 1983. The task force proposed two terms for vulvar pain vulvodynia and burning vulva syndrome. Young AW Jr, Azoury R, McKay M. Burning vulvar syndrome. Report of the ISSVD taskforce. J Reprod Med 1984 29:457.
1987 Friedrich reported on 86 patients who were treated for what he called vulvar vestibulitis syndrome. These women presented with:
1) severe pain on vestibular touch or attempted vaginal entry
2) tenderness to pressure localized with the vulvar vestibule
3) physical findings confined to vestibular erythema of various degrees.
The classic patient with pure vulvar vestibulities syndrome does not have symptoms unless the area is touched or manipulated, for example when sexual relations are attempted. The patient only experiences burning or discomfort when pressure is applied to the vulva.
The author was of the opinion that the term vulvar vestibulitis syndrome should be adopted as the standard description of this disorder.
//Friedrich EG Jr. Vulvar vestibulitis syndrome. J Reprod Med 1987 32:110-114.//
However, in 1997, the term vestibulodynia was introduced by Dr Jacob Bornstein et al in a study designed to recognize special features of women with both vestibulitis and constant vulvar pain (vestibulodynia) and to determine whether vestibulodynia is a separate disease entity or a subset of vestibulitis.
//Bornstein J, Zarfati D, Goldschmid N, Stolar Z, Lahat N, Abramovici H. Vestibulodynia – a subset of vulvar vestibulitis or a novel syndrome. Am J Obstet Gynecol. 1997 177:1439-1443.//
1999 The ISSVD came up with a proposal for Terminology and Classification during the 1999 ISSVD World Congress. It was argued that the suffix “-itis” wrongly implied that vestibulitis was an inflammatory disorder. While the membership narrowly voted to accept changes in terminology and a new classification, many members were not happy with this new classification:
VULVAR DYSESTHESIA (ISSVD 1999)
Generalized Vulvar Dysesthesia (formerly dysesthetic vulvodynia)
Localized Vulvar Dysesthesia
  • Vestibulodynia (formerly vulvar vestibulitis)
  • Clitorodynia
  • Other localized forms of vulvar dysesthesia
2001 At the following ISSVD World Congress, the members preferred to divide the subsets of vulvar dysesthesia by stimulus rather than by site:
VULVAR DYSESTHESIA (ISSVD 2001, Provisional)
Provoked vulvar dysesthesia
  • Generalized
  • Localized (vestibule, clitoris, other)
Spontaneous vulvar dysesthesia
  • Generalized
  • Localized (vestibule, clitoris, other)
Since there was still much disagreement on terminology and classification, Drs Lynch and Moyal-Barraco were invited by the ISSVD to develop new proposals with multi-stakeholder input. They proposed that the term vulvodynia should be reinstated “because it was indexed in the International Classification of Disease (ICD-9) and because it was a term widely used and accepted by patients and physicians.” They also proposed that “dysesthesia” be deleted. The proposals were submitted to the membership for comment and these comments were incorporated into the 2003 ISSVD Proposal for Terminology and Classification.
2003 At the 17th ISSVD World Congress in 2003, “the membership voted almost unanimously to return to the use of the popular and widely used term “vulvodynia” for vulvar pain occurring in the absence of an underlying recognisable disease., to accept a modified definition of vulvodynia and to classify the subsets of vulvodynia on the basis of the site involved.
ISSVD Terminology and Classification of Vulvar Pain (2003):
A) Vulvar Pain Related to a Specific Disorder
1) Infectious (e.g. candidiasis, herpes, etc.)
2) Inflammatory (e.g. lichen planus, immunobullous disorders, etc.)
3) Neoplastic (e.g. Paget’s disease, squamous cell carcinoma, etc.)
4)Neurologic (e.g. herpes neuralgia, spinal nerve compression, etc.)
B) Vulvodynia
1) Generalized
a) Provoked (sexual, nonsexual, or both)
b) Unprovoked
c) Mixed (provoked and unprovoked)
2) Localized (vestibulodynia, clitorodynia, hemivulvodynia, etc.)
a) Provoked (sexual, nonsexual, or both)
b) Unprovoked
c) Mixed (provoked and unprovoked)
DEFINITIONS AND NOTES
“Vulvodynia” is 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. Specifically, a peripheral neuropathy (e.g. related to herpes zoster or herpes simplex) should be excluded based on the lack of associated symptoms such as sphincter dysfunction, weakness in the lower limbs, or sensory changes such as hypoesthesia or anesthesia involving the area of discomfort. Vulvodynia is represented under the term “vulvar pain syndrome” in the classification of the International Society for the Study of Pain.
“Relevant” takes into account the following considerations: 1) diffuse and periductal vestibular erythema (i.e. bilateral, usually symmetrical, erythema localized around the openings of Bartholin’s glands and minute epithelial depressions) is a normal finding, and is therefore not responsible for vulvar discomfort; 2) disorders such as genital warts, nevi, cyst, etc. may be present on the vulva but not be relevant (i.e. not necessarily responsible for vulvar discomfort).
“Generalized” specifies involvement of the whole vulva and “localized” specifies involvement of a portion of the vulva such as the vestibule (vestibulodynia), the clitoris (clitorodynia), the hemivulva (hemivulvodynia) etc.
“Unprovoked” means that the discomfort occurs spontaneously without a specific physical trigger; “provoked” means that the discomfort is triggered by physical contact. Such contact may be sexual, nonsexual or both. Examples include intromission, clothing pressure, tampon insertion, cotton tip applicator pressure, fingertip pressure, etc.
“Vestibulitis” has been deleted from the ISSVD terminology because the presence of inflammation, as implied by the suffix “-itis”, has not so far been documented. The term vestibulitis is now replaced by “provoked vestibulodynia” defined as discomfort on intromission (introital dyspareunia), clothing pressure, tampon insertion, cotton tip applicator pressure, fingertip pressure, etc.
Source:
Moyal-Barracco M, Lynch PJ.//2003 ISSVD terminology and classification of vulvodynia: a historical perspective.// J Reprod Med. 2004 Oct;49(10):772-7.
2006 Bachmann GA, Rosen R, Pinn VW, Utian WH, Ayers C, Basson R, Binik YM, Brown C, Foster DC, Gibbons JM Jr, Goldstein I, Graziottin A, Haefner HK, //Harlow L//, Spadt SK, Leiblum SR, Masheb RM, Reed BD, Sobel JD, Veasley C,Wesselmann U, Witkin SS. //Vulvodynia: a state-of-the-art consensus on definitions, diagnosis and management.// J Reprod Med. 2006 Jun;51(6):447-56.
This "white paper" describes the findings and recommendations of a consensus conference panel based on a comprehensive review of the published literature on vulvodynia in addition to expert presentations on research findings and clinical management approaches.
2007 Haefner, HK. //Report of the International Society for the Study of Vulvovaginal Disease Terminology and Classification of Vulvodynia//. J Low Gen Tract Dis. 2007; 11(1):48-9.
In 2007, Haefner writes: Vulvodynia has been redefined by the International Society for the Study of Vulvovaginal Disease as vulvar discomfort in the absence of gross anatomic or neurologic findings. Classification is based further on whether the pain is generalized or localized and whether it is provoked, unprovoked, or both.
2010
http://www.issvd.org/document_library/BibliographyTerminology2010.pdf
This link provides the most up-to-date ISSVD official terminology, including vulval pain.
Refs.
Moyal-Barracco M, Lynch PJ.//2003 ISSVD terminology and classification of vulvodynia: a historical perspective.// J Reprod Med. 2004 Oct;49(10):772-7.
Haefner, HK. //Report of the International Society for the Study of Vulvovaginal Disease Terminology and Classification of Vulvodynia//. J Low Gen Tract Dis. 2007; 11(1):48-9.
2011 National Vulvodynia Association www.nva.org. In 2011, the National Vulvodynia Association published a summary of the most current Definitions and Types of Vulvodynia in NVA News, Volume XVI, Issue II, Summer 2011, as follows:
Vulvodynia is chronic (more than three to six months) vulvar pain without an identifiable cause. The location, constancy and severity of the pain vary among women. The two main subtypes of vulvodynia, which sometimes co-exist, are:
Provoked Vestibulodynia (PVD) (Previously: Vulvar Vestibulitis Syndrome)
Women with PVD have pain limited to the vestibule, the area surrounding the opening of the vagina, that occurs during/after touch or pressure, e.g. with intercourse, tampon insertion and/or prolonged sitting. PVD is further classified as primary pain (pain since the first attempt at vaginal penetration) or secondary pain (pain that starts after a period of pain-free vaginal penetration).
Generalized Unprovoked Vulvodynia (GV) (Previously Dysesthetic or Essential Vulvodynia)
Women with GV have spontaneous pain in multiple areas of the vulva. It is relatively constant, but there can be some periods of symptom relief. Activities that apply pressure to the vulva, such as prolonged sitting or simply wearing pants, typically exacerbate symptoms.
For further information from the NVA on terminology, go to: http://learnprovider.nva.org/terminology_classification.html
Book CLINICAL MANAGEMENT OF VULVODYNIA – TIPS AND TRICKS. Authors: Alessandra Graziottin, Filippo Murina. Publisher: Springer-Verlag, 2011, hardcover, pp. 87, ISBN: 978-88-470-1925-6- Includes an interesting and controversial discussion on names and definitions , noting that until now vulvodynia has been used to describe unexplained vulvar pain. The authors suggest that the term vulvodynia “should include all types of vulvar pain

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