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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 l.jpg

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 l.jpg
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 l.jpg
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 l.jpg
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 l.jpg
    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 l.jpg
    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 l.jpg
    Results from a self-report survey of vulvodynia patients administered by the National Vulvodynia Association


    Differential diagnosis and treatment of vulvar pain l.jpg

    Differential Diagnosis and Treatment of Vulvar Pain administered by the National Vulvodynia Association


    Dysesthesia l.jpg
    Dysesthesia administered by the National Vulvodynia Association

    • 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 l.jpg
    Subjective Findings administered by the National Vulvodynia Association

    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 l.jpg
    Objective Findings administered by the National Vulvodynia Association

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

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


    Vulvar dermatoses l.jpg

    Vulvar Dermatoses administered by the National Vulvodynia Association


    Lichen simplex chronicus lsc l.jpg

    Lichen Simplex Chronicus administered by the National Vulvodynia Association(LSC)


    Lsc general information l.jpg
    LSC: General Information administered by the National Vulvodynia Association

    • 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 l.jpg
    LSC: Diagnosis administered by the National Vulvodynia Association

    • 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 l.jpg
    LSC – Classic Presentation administered by the National Vulvodynia Association

    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 l.jpg
    LSC: Treatment administered by the National Vulvodynia Association

    • 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


    Lichen sclerosus ls l.jpg

    Lichen Sclerosus administered by the National Vulvodynia Association(LS)


    Ls general information l.jpg
    LS: General Information administered by the National Vulvodynia Association

    • 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 l.jpg
    LS: Diagnosis administered by the National Vulvodynia Association

    • 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 l.jpg
    LS – Classic Presentation administered by the National Vulvodynia Association

    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 l.jpg
    LS – Subtle Presentation administered by the National Vulvodynia Association

    Occasionally, very mild

    lichen sclerosus, such as

    the faint white spots on

    the left side of the photo,

    can cause pain.


    Ls treatment l.jpg
    LS: Treatment administered by the National Vulvodynia Association

    • 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


    Lichen planus lp l.jpg

    Lichen Planus administered by the National Vulvodynia Association(LP)


    Lp diagnosis l.jpg
    LP: Diagnosis administered by the National Vulvodynia Association

    • 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 l.jpg
    LP: Classic Presentation administered by the National Vulvodynia Association

    Lichen planus with

    irregular white lines is

    classic, and the deep red

    areas are painful

    erosions.


    Lp subtle presentation l.jpg
    LP: Subtle Presentation administered by the National Vulvodynia Association

    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 l.jpg
    LP: Treatment administered by the National Vulvodynia Association

    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 l.jpg
    Additional Differential Diagnoses administered by the National Vulvodynia Association

    • 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 l.jpg
    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


    Redneck yacht l.jpg
    Redneck Yacht treatments


    Vulvar vestibulitis syndrome vvs l.jpg

    Vulvar Vestibulitis Syndrome treatments(VVS)

    Also known as:

    localized vulvar dysesthesia

    vestibulodynia


    Vvs general information l.jpg
    VVS: General Information treatments

    • 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 l.jpg
    Anatomy of the Vestibule treatments

    • 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 l.jpg
    Vulvar Diagram treatments

    Diagram from The Vulvodynia Survival Guide, reproduced

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


    Vvs diagnosis l.jpg
    VVS: Diagnosis treatments

    • 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 l.jpg
    VVS: Clinical Presentation treatments

    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 l.jpg
    Levels of dyspareunia used to stratify severity of VVS treatments

    • 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 l.jpg
    Pathophysiology of VVS treatments

    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 l.jpg
    Pathophysiology of VVS treatments

    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 l.jpg
    Pathophysiology of VVS treatments

    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 l.jpg
    Pathophysiology of VVS treatments

    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 l.jpg
    VVS: Treatment treatments

    • 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 l.jpg

    Dysesthetic Vulvodynia treatments(DVY)

    Also known as:

    “essential” vulvodynia

    generalized vulvar dysesthesia


    Dvy general information l.jpg
    DVY: General Information treatments

    • 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 l.jpg
    DVY: Presentation and Diagnosis treatments

    • 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 l.jpg
    DVY: Possible Pathophysiologic Mechanisms treatments

    • 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 l.jpg
    DVY: Treatment treatments

    • 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


    Resources l.jpg

    Resources treatments


    Slide54 l.jpg

    • Associations treatments

      • 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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