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Vulvodynia . Howard A. Shaw, M.D. Chairman/Director Department of Obstetrics and Gynecology St. Francis Hospital and Medical Center Hartford, CT. Redneck Jacuzzi. Early Descriptions: Hyperaesthesia of the Vulva. 1880

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vulvodynia

Vulvodynia

Howard A. Shaw, M.D.

Chairman/Director

Department of Obstetrics and Gynecology

St. Francis Hospital and Medical Center

Hartford, CT

early descriptions hyperaesthesia of the vulva
Early Descriptions: Hyperaesthesia of the Vulva

1880

“…excessive sensibility of the nerves supplying the mucous membrane of some portion of the vulva; sometimes…confined to the vestibule…other times to one labium minus…”

Thomas, T.G., Practical Treatise on the Diseases of Women,

Henry C. Lea’s Son & Co., Philadelphia, 1880, pp. 145-147.

1888

“This disease…is characterized by a supersensitiveness of the vulva…No redness or other external manifestation of the disease is visible…When…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…Sexual intercourse is equally painful, and becomes in aggravated cases impossible.”

Skene, A.J.C., Diseases of the external organs of generation,

In: Treatise on the Diseases of Women, New York, D. Appleton and Co., 1888, 77-99.

definition
Definition

International Society for the Study of

Vulvovaginal Disease (ISSVD)

1983 World Congress

  • Chronic vulvar discomfort, especially that characterized by the patient’s complaint of burning (and sometimes stinging, irritation or rawness)
  • Symptoms may have multiple causes

Young, A.W., Azoury, R.S., McKay, M., Pincus, S., Ridley, C.M. and Zerner, J., Burning vulva syndrome: report of ISSVD task force, Journal of Reproductive Medicine 29 (1984) 457.

nomenclature
Nomenclature

Subtypes of Vulvodynia:

  • Vulvar Vestibulitis Syndrome (VVS)

also known as:

      • Vestibulodynia
      • localized vulvar dysesthesia
  • Dysesthetic Vulvodynia

also known as:

      • “essential” vulvodynia
      • generalized vulvar dysesthesia
preliminary data
Preliminary Data
  • 480 women surveyed, 20-59 years of age
  • 303 (70%) returns
  • 56 (18.5%) reported a history of genital tract discomfort persisted for greater than 3 months
    • Of these women, 39% never sought treatment
    • Of those who sought treatment, the condition remained undiagnosed in 38%
  • 12% had pain on contact
  • 6% had persistent pain or itching

Harlow, B.L., Wise, L.A.and Stewart, E.G., Prevalence and predictors of chronic lower

genital tract discomfort, American Journal of Obstetrics and Gynecology, 185 (2001) 545-50.

5 year ongoing study
5-year Ongoing Study
  • 16,000 women from the greater Boston area are being surveyed via a mailed questionnaire
  • 2nd screening telephone questionnaires administered to those thought to have vulvodynia
  • 20% of those who meet the criteria for vulvodynia will be examined by a specialist in the field
  • Cases will be identified and matched with controls
    • venous blood, vaginal lavage, vulvar swab specimens, etc., assessing cytokines and microbiological organisms

Harlow, B.L., Project Title: Prevalence and Etiological Predictors of Vulvodynia,

NIH Grant Number: 1R01HD038428-01A1

slide10
Results from a self-report survey of vulvodynia patients administered by the National Vulvodynia Association
dysesthesia
Dysesthesia
  • Unpleasant, abnormal sensation
    • examples include:
      • burning
      • rawness
  • Can be spontaneous or evoked
  • Includes allodynia and/or hyperalgesia
    • Allodynia:

Pain due to a stimulus that does not normally evoke pain

    • Hyperalgesia:

Increased response to a stimulus that IS normally painful

subjective findings
Subjective Findings

Symptoms can be constant or intermittent, spontaneous or evoked:

  • Pain
  • Burning (can be constant and severe)
  • Rawness
  • Irritation
  • Dryness
  • Hyperpathia (pain provoked by very light touch)
objective findings
Objective Findings

Turner, M.L.C. and Marinoff, S.C., General principles in the diagnosis and

treatment of vulvar diseases, Dermatologic Clinics, 10 (1992) 275-281.

lsc general information
LSC: General Information
  • End stage of itch-scratch-itch cycle in predisposed patients due to:
    • Irritants
    • Infections
    • VIN
  • Patients often frustrated by long course of symptoms and having seen many physicians
  • Recurrence is common
lsc diagnosis
LSC: Diagnosis
  • Patient reports intense pruritus with relief upon scratching
  • Thick, lichenified skin – often reddened
  • May exhibit erosions or fissuring
  • Culture for yeast and bacteria
lsc classic presentation
LSC – Classic Presentation

Usually, the skin

abnormalities of lichen

simplex chronicus (aka

eczema, atopic dermatitis,

neurodermatitis) are caused

by rubbing or scratching, as

can be seen from the rubbed

and thickened skin in this

woman.

lsc treatment
LSC: Treatment
  • Remove irritants or allergens (if known) and stop all topicals, soaps, douches, etc.
  • Sitz baths or compresses 1-2x/day for 10-15 minutes (before application of steroids)
  • Mid-to-high potency topical corticosteroid
    • Clobetasol 0.05% daily or
    • Triamcinolone 0.1% bid
  • Counsel patient about vulvar self-care measures to minimize risk of recurrence
  • Treat any underlying infection
ls general information
LS: General Information
  • Etiology unknown, generally believed to be autoimmune
  • Occurs on genital skin in about 80% of cases
  • Females of any age can develop LS, including young children, toddlers and infants (as can males) but most symptomatic are post-menopausal women
  • Childhood LS can resolve at puberty (children should be followed very carefully throughout adolescence – do not assume that no symptoms equals no disease)
  • Sometimes improves during pregnancy (usually 2nd tri)
  • Often misdiagnosed as yeast infections, herpes or vitiligo
  • 2-5% risk of developing vulvar squamous cell carcinoma
ls diagnosis
LS: Diagnosis
  • Pathognomonic sign is texture change – crinkling, occasionally looks waxy
  • Punch biopsy typically used
    • in women with severely fragile skin or in children, treatment is sometimes initiated without a biopsy
  • Histological findings:
    • hallmark is liquefaction degeneration of the basal cell layer with homogenization of collagen in the dermis (epidermis can be atrophic or thickened)
  • Hypo-pigmentation – “butterfly” or “keyhole” appearance
  • Pruritus, sometimes burning or pain
  • Atrophy and increased risk of fissures
  • In advanced or untreated cases: clitoral hood fuses; labia minora fused to majora; narrowing of the introitus; dyspareunia
ls classic presentation
LS – Classic Presentation

Severe lichen sclerosus

is itchy and it can be

identified by the white

color and easy bruising

and tearing when rubbed,

obviously a cause of

symptoms.

ls subtle presentation
LS – Subtle Presentation

Occasionally, very mild

lichen sclerosus, such as

the faint white spots on

the left side of the photo,

can cause pain.

ls treatment
LS: Treatment
  • Topical clobetasol propionate 0.05% 1-2x/day
    • Reduce frequency and/or potency when texture and/or symptoms normalize
  • Testosterone and progesterone do not work better than petrolatum ointment (Vaseline) alone
  • Dilator and/or sex therapy may be helpful for women who experience dyspareunia
    • First treat the vulvar skin to help restore elasticity – and recommend using lubrication
  • Counsel patient on vulvar self-care measures
  • Skin grafting not recommended due to high rate of recurrence
lp diagnosis
LP: Diagnosis
  • Differentiating LS & LP can be difficult; can also co-exist
  • A biopsy is helpful in diagnosing LP but histological findings are sometimes non-specific
  • May be associated with slightly increased risk of cancer
  • Histological findings:
    • Hallmark is a dense chronic inflammatory infiltrate hugging and obscuring the basal cell layer with occasional necrotic keratinocytes
  • Classic Non-erosive Lichen Planus
    • white lacy or fern-like papules
  • Erosive Lichen Planus
    • Clearly demarcated red plaques on oral and/or genital membranes with white “lacy” edges
    • Erythematous lesions in the vestibule & up into vagina
    • Burning pain; dyspareunia
    • May resemble lichen sclerosus, particularly when late agglutination of architecture occurs
lp classic presentation
LP: Classic Presentation

Lichen planus with

irregular white lines is

classic, and the deep red

areas are painful

erosions.

lp subtle presentation
LP: Subtle Presentation

Even subtle lichen

planus can hurt, as it

does in this woman who

has mild white

streakiness towards the

posterior fourchette, and

small posterior vestibular

erosions.

lp treatment
LP: Treatment

Options include:

  • Ultrapotent corticosteroids with careful follow-up for vulva; hydrocortisone foam for vagina
  • Hydroxychloroquine
  • Anti metabolites
  • Systemic retinoids
  • Vaginal dilator therapy for women with introital stenosis and/or labial adhesions
additional differential diagnoses
Additional Differential Diagnoses
  • Fungal and bacterial infections
  • Contact dermatitis
  • Human Papillomavirus (HPV)
  • Vaginitis
  • Psoriasis
  • Ulcers
  • Paget’s disease
  • Herpes simplex virus
  • Condyloma Acuminata
  • Vulvar Intraepithelial Neoplasia (VIN)
  • Vulvar Cancer
suggestions for instructing patients in applying topical treatments
Suggestions for instructing patients in applying topical treatments

Some topical treatments are very effective, however caution should be used in their application.

Give specific instructions for applying topical treatments for the vulva:

  • Amount of cream
    • Squeeze correct amount of treatment sample on your own finger during office visit
  • Application site
    • Some women will have never seen their vulva
    • Shade in or point to areas on a vulvar diagram to indicate correct application site
    • Have patient apply treatment during visit, using a mirror for clarity
vulvar vestibulitis syndrome vvs

Vulvar Vestibulitis Syndrome(VVS)

Also known as:

localized vulvar dysesthesia

vestibulodynia

vvs general information
VVS: General Information
  • Etiology unknown
  • Average VVS patient is in her 30s
  • Not psychogenic
  • Can co-exist with dysesthetic vulvodynia (generalized vulvar dysesthesia)
  • Use caution when diagnosing and treating concurrent conditions
    • treatments for yeast, BV and HPV can all worsen VVS (as well as other vulvar disorders)
anatomy of the vestibule
Anatomy of the Vestibule
  • frenulum of clitoris anteriorly to the forchette posteriorly
  • inner most border is hymeneal ring
  • lateral border is Hart’s line on the inner aspect of the labia minora
  • contains major vestibular glands (Bartholin’s, Skene’s and periurethral) and minor vestibular glands
  • derived from urogenital sinus endoderm
vulvar diagram
Vulvar Diagram

Diagram from The Vulvodynia Survival Guide, reproduced

with permission of author, Howard I. Glazer, Ph.D.

vvs diagnosis
VVS: Diagnosis
  • Rule out infection, dermatoses (biopsy or colposcopy may be necessary) and any other cause of pain
  • Diagnosed using Friedrich’s Criteria:
    • Severe pain on vestibular touch or attempted vaginal entry
    • Tenderness to pressure localized within the vulvar vestibule
    • No evidence of physical findings except for varying degrees of erythema

Friedrich Jr., E.G., Vulvar vestibulitis syndrome,

Journal of Reproductive Medicine, 32 (1987) 110-114.

vvs clinical presentation
VVS: Clinical Presentation

This patient shows minimal erythema of the left vestibule that may be normal for this patient. However, there is more obvious redness at the opening of the vestibular gland (arrow).

levels of dyspareunia used to stratify severity of vvs
Levels of dyspareunia used to stratify severity of VVS
  • 0: No dyspareunia
  • 1: Causes discomfort but does not interfere

with frequency of intercourse

  • 2: Sometimes prevents intercourse
  • 3: Completely prevents intercourse
pathophysiology of vvs
Pathophysiology of VVS

Vestibular Nerve Fiber Proliferation in Vulvar Vestibulitis Syndrome

Westrom, L.V. and Willen, R., Obstetrics and Gynecology, 91 (1998) 572-576.

FINDINGS

Vestibular neural hyperplasia may provide a morphologic explanation of the pain in vulvar vestibulitis syndrome.

pathophysiology of vvs44
Pathophysiology of VVS

Increased Intraepithelial Innervation in Women with Vulvar Vestibulitis Syndrome

Bohm-Starke, N., Hilliges, M., Falconer, C. and Rylander E.,

Gynecologic and Obstetric Investigation, 46 (1998) 256-260.

FINDINGS

Nerve supply in the vestibular mucosa in women with VVS and those free of symptoms were studied by PGP 9.5 immunohistochemistry. There was a significant increase in the number of intraepithelial nerve endings in women with VVS indicating an alteration in the nerve supply.

pathophysiology of vvs45
Pathophysiology of VVS

Elevated Tissue Levels of Interleukin-1β and Tumor Necrosis Factor-α in Vulvar Vestibulitis

Foster, D.C. and Hasday, J.D., Obstetrics and Gynecology, 89 (1997) 291-6.

FINDINGS

Concentrations of IL-1β and TNF-α were elevated in women with vulvar vestibulitis relative to those in asymptomatic controls. This elevation varied according to anatomic site. Inflammatory cytokine elevation may contribute to the pathophysiology of mucocutaneous hyperalgesia.

pathophysiology of vvs46
Pathophysiology of VVS

Interleukin-1 Receptor Antagonist Gene Polymorphism in Women with Vulvar Vestibulitis

Jeremias, J., Ledger, W.J. and Witkin, S.S., American

Journal of Obstetrics and Gynecology. 182 (2000) 283-5.

FINDINGS

Polymorphisms in the gene coding for the interleukin-1 receptor antagonist, a naturally occurring down-regulator of proinflammatory immune response, were studied. The unique distribution of interleukin-1 receptor antagonist alleles among women with VVS suggests that polymorphism in this gene may be a factor influencing susceptibility to this syndrome, severity of symptoms, or both.

vvs treatment
VVS: Treatment
  • Eliminate irritants
  • Counsel patient on vulvar self-care and self-help tips
  • Topical estradiol may decrease severity of symptoms
  • Topical anesthetics (e.g. lidocaine)
  • Pelvic floor therapy (for those who have pelvic floor muscle abnormalities as measured by surface electromyography)
  • Physical therapy
  • Interferon injections
  • Surgery (vestibulectomy with vaginal advancement) usually used after more conservative therapies are exhausted (high success rates of 70%+)
  • Tricyclic antidepressants (e.g. amitriptyline) or anti-convulsants (e.g. neurontin) may be helpful for their pain-blocking qualities
  • CO2 LASER VAPORIZATION NO LONGER RECOMMENDED
dysesthetic vulvodynia dvy

Dysesthetic Vulvodynia(DVY)

Also known as:

“essential” vulvodynia

generalized vulvar dysesthesia

dvy general information
DVY: General Information
  • Etiology unknown
  • Average age of patient is 40s and above, but women of all ages can be affected
  • Not psychogenic
  • Can co-exist with vulvar vestibulitis syndrome (vulvar dysesthesia localized to the vestibule)
  • Use caution when diagnosing and treating concurrent conditions
    • treatments for yeast, BV and HPV can all worsen vulvodynia

(as well as other vulvar disorders)

dvy presentation and diagnosis
DVY: Presentation and Diagnosis
  • Presentation:
    • Erythema may or may not be present
    • Pain can be intermittent or constant
    • Patients may experience periods of unexplained relief and/or flares of pain
    • Symptoms may be similar to a UTI (e.g. frequency, urgency, dysuria) with negative urine cultures
  • Diagnosis:
    • Diagnosis of exclusion
    • Rule out infection, dermatoses (biopsy or colposcopy may be necessary) and any other cause for pain
    • Test for allodynia, hypo- or hyperalgesia using cotton swab test or vulvoalgesiometer
dvy possible pathophysiologic mechanisms
DVY: Possible Pathophysiologic Mechanisms
  • Believed to be a neuropathic pain syndrome
  • Pudendal nerve intrapment
  • Pudendal nerve injury from childbirth, previous surgery or a wide variety of other insults (horseback riding, bicycling, sports trauma, etc.)
  • Referred pain from ruptured disc or scarring around sacral nerve roots after disc surgery
  • Sacral-meningeal (Tarlov’s) cysts
  • Referred pain from pelvic floor musculature dysfunction or orthopedic condition affecting these muscles
  • Neuropathic viruses – varicella zoster, herpes simplex may lead to post-herpetic neuralgia manifesting as VDY
  • Neurologic disease (such as multiple sclerosis, etc.)
dvy treatment
DVY: Treatment
  • Eliminate any irritants
  • Counsel patient on vulvar self-care and self-help tips
  • Any vaginal atrophy should be treated with estradiol
  • Topical local anesthetics (e.g. lidocaine) may be helpful
  • Tricyclic antidepressants (e.g. amitriptyline, etc.)
  • Anticonvulsants (e.g. Neurontin, etc.)
  • Refer to specialist (if appropriate):
    • Nerve blockade and/or other pain management strategies
    • Pelvic floor therapy (for those who have pelvic floor muscle abnormalities as measured by surface electromyography)
    • Physical therapy
  • SURGERY NOT INDICATED
slide54
Associations
    • National Vulvodynia Association

www.nva.org or 301-299-0775

      • Services for patients and health care professionals
    • International Society for the Study of Vulvovaginal Disease

www.issvd.org or 704-814-9493

  • Books
    • The V Book: A Doctor’s Guide to Complete Vulvovaginal Health

by Elizabeth Gunter Stewart, MD and Paula Spencer

    • The Vulvodynia Survival Guide: How to Overcome Painful Vaginal Symptoms & Enjoy an Active Lifestyle

by Howard I. Glazer, Ph.D. and Gae Rodke, M.D.

  • Internet
    • www.nva.org
    • www.vulvarhealth.org
    • www.vulvodynia.com
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