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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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    1. Vulvodynia Howard A. Shaw, M.D. Chairman/Director Department of Obstetrics and Gynecology St. Francis Hospital and Medical Center Hartford, CT

    2. Redneck Jacuzzi

    3. 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.

    4. 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.

    5. 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

    6. NIH Funded Prevalence Study: Harvard University

    7. 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.

    8. 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

    9. Coexisting Medical Conditions

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

    11. Differential Diagnosis and Treatment of Vulvar Pain

    12. 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

    13. 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)

    14. 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.

    15. Vulvar Dermatoses

    16. Lichen Simplex Chronicus(LSC)

    17. 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

    18. 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

    19. 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.

    20. 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

    21. Lichen Sclerosus(LS)

    22. 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

    23. 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

    24. 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.

    25. LS – Subtle Presentation Occasionally, very mild lichen sclerosus, such as the faint white spots on the left side of the photo, can cause pain.

    26. 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

    27. Lichen Planus(LP)

    28. 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

    29. LP: Classic Presentation Lichen planus with irregular white lines is classic, and the deep red areas are painful erosions.

    30. 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.

    31. 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

    32. 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

    33. 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

    34. Redneck Yacht

    35. Vulvar Vestibulitis Syndrome(VVS) Also known as: localized vulvar dysesthesia vestibulodynia

    36. 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)

    37. 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

    38. Vulvar Diagram Diagram from The Vulvodynia Survival Guide, reproduced with permission of author, Howard I. Glazer, Ph.D.

    39. 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.

    40. 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). ◄

    41. 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

    42. 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.

    43. 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.

    44. 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.

    45. 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.

    46. 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

    47. Dysesthetic Vulvodynia(DVY) Also known as: “essential” vulvodynia generalized vulvar dysesthesia

    48. 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)

    49. 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