Localized Vulvodynia


Introduction

Vulvodynia is defined as a chronic discomfort or pain, consisting of burning, stinging, irritation, and rawness on the vulva. It was once believed to be a rare condition, but is now known to affect millions of women. It can be a chronic pain disorder, at times associated with other chronic comorbid pain conditions. The prevalence of vulvodynia is 8.3%. The prevalence remains stable through age 70 years and has been shown to decline thereafter. The pain can be generalized—diffuse vulvar burning or irritation—or localized—pain at a specific area, such as the vestibule or clitoris. Localized vulvodynia that was previously termed vulvar vestibulitis is currently termed vestibulodynia . The vestibule is defined as the area between the hymen and Hart's line ( Fig. 3.1 ).

Fig. 3.1, Diagram of the vulvar vestibule.

History of Vulvar Pain Terminology

Vulvar pain discussion first appeared in the literature in 1861 in an article by Sims. He described a patient he saw in 1857 with vaginismus, but on further analysis of her history, she appeared to have vulvodynia. Thomas has described the condition as “excessive hypersensibility of the nerves supplying the mucous membrane of some portion of the vulva.” In 1889, Skene depicted a condition “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.” In the same year, Kellogg wrote about a patient with “sensitive points about the mouth of the vagina.” The topic was not readdressed until 1928, when Kelly saw a woman with “exquisitely sensitive deep red spots” that were a “fruitful source of dyspareunia.” In 1983, Friedrich reported on 13 patients with “vestibular adenitis.” Terminology has continued to change with time. The International Society for the Study of Vulvovaginal Disease (ISSVD) popularized a definition of vulvar pain in the 1980s (essential or dysesthetic vulvodynia), describing patients with a chronic discomfort and burning, stinging, irritation, and rawness of the vulva. In 1987, Friedrich proposed the term vulvar vestibulitis syndrome . There are two major forms of vulvar pain: hyperalgesia (low pain threshold) and allodynia (pain to light touch). The terminology of pain localized to the vulvar vestibule continues to undergo changes. Table 3.1 demonstrates the changes in terminology for vulvar pain that have occurred with time, up to the 2003 terminology. Concerns with the terminology were present. Recently, a new vulvodynia terminology has been approved ( Box 3.1 ).

Table 3.1
ISSVD Terminology and Classification for Vulvodynia
Vulvar Dysesthesia (1999) Vulvar Dysesthesia (2001) Vulvodynia (2003)
New Mexico, United States, ISSVD World Congress Lisbon, Portugal, ISSVD World Congress
(N ote : This was a provisional terminology system.)
Salvador, Brazil, ISSVD World Congress
Generalized vulvar dysesthesia Provoked vulvar dysesthesia
Generalized
Localized (vestibule, clitoris, other)
Vulvodynia, generalized
Provoked (sexual, nonsexual, both)
Unprovoked
Mixed (provoked and unprovoked)
Localized vulvar dysesthesia
Vestibulodynia (formerly vulvar vestibulitis)
Clitorodynia
Other localized forms of vulvar dysesthesia
Spontaneous vulvar dysesthesia
Generalized
Localized (vestibule, clitoris, other)
Vulvodynia, localized (vestibulodynia, clitorodynia, hemivulvodynia)
Provoked (sexual, nonsexual, both)
Unprovoked
Mixed (provoked and unprovoked)
ISSVD, The International Society for the Study of Vulvovaginal Disease.

Box 3.1
From Bornstein J, Goldstein AT, Stockdale CK, et al: 2015 ISSVD, ISSWSH, and IPPS consensus terminology and classification of persistent vulvar pain and vulvodynia. J Low Genit Tract Dis 20:126–130, 2016.
2015 ISSVD, ISSWSH, and IPPS Consensus Terminology and Classification of Persistent Vulvar Pain and Vulvodynia

  • A.

    Vulvar pain caused by a specific disorder a

    a Women may have both a specific disorder (e.g., lichen sclerosus) and vulvodynia.

    • Infectious (e.g., recurrent candidiasis, herpes)

    • Inflammatory (e.g., lichen sclerosus, lichen planus, disorders)

    • Neoplastic (e.g., Paget disease, squamous cell carcinoma)

    • Neurologic (e.g., postherpetic neuralgia, nerve compression or injury, neuroma)

    • Trauma (e.g., female genital cutting, obstetric)

    • Iatrogenic (e.g., postoperative, chemotherapy, radiation)

    • Hormonal deficiencies (e.g., genitourinary syndrome of menopause [vulvovaginal atrophy], lactational amenorrhea)

  • B.

    Vulvodynia—vulvar pain of at least 3 months' duration, without clear identifiable cause, which may have potential associated factors

    The following are the descriptors:

    • Localized (e.g., vestibulodynia, clitorodynia) or generalized or mixed (localized and generalized)

    • Provoked (e.g., insertional, contact), spontaneous, or mixed (provoked and spontaneous)

    • Onset (primary or secondary)

    • Temporal pattern (intermittent, persistent, constant, immediate, delayed)

IPPS, International Pelvic Pain Society; ISSWSH, The International Society for the Study of Women's Sexual Health

ISSVD, The International Society for the Study of Vulvovaginal Disease.

Patients with vulvar pain localized to the vestibule have a normal-appearing vulva, other than occasional erythema ( Fig. 3.2 ). The erythema tends to be most prominent at the Bartholin's, Skene's, and vestibular duct openings. The other form of localized pain is clitorodynia. Pain associated with an abnormal vulvar appearance does not qualify for the diagnosis of localized vulvodynia ( Box 3.2 ).

Fig. 3.2, A, B, Clinical photographs of localized vulvodynia (vestibulodynia) showing introital erythema.

Box 3.2
Diseases Associated With Vulvar Pain, Not Qualifying for the Diagnosis of Vulvodynia

  • Podophyllin overdose

  • Condylox (podofilox) overdose

  • Behçet disease

  • Aphthous ulcers

  • Herpes (simplex and zoster)

  • Candidiasis

  • Trichomonas

  • Chancroid

  • Sjögren disease

  • Contact dermatitis

  • Endometriosis

  • Pemphigus

  • Pemphigoid

  • Atrophy

  • Lichen sclerosus

  • Lichen planus

  • Crohn disease

  • Bartholin's abscess

  • Trauma

  • Imperforate hymen

  • Prolapsed urethra

  • Vulvar intraepithelial neoplasia

  • Carcinoma

Causative Theories of Vulvodynia

Many theories have been proposed for the causes of vulvodynia, including abnormalities of embryologic development, infection, inflammation, genetics, immune factors, nerve sensitization, and increased muscle tone. The exact cause of vulvodynia is unknown; there likely is no single cause.

Embryologic Development

McCormack and Fitzpatrick et al. have linked vestibular pain with interstitial cystitis, another idiopathic inflammatory syndrome of the urogenital tract. They have speculated that the tissues from two distinct anatomic sites have a common embryologic origin and therefore are predisposed to similar pathologic responses when challenged. Additional studies have found an association between vulvodynia and interstitial cystitis.

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