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
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,

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
“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)

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: 1R01HD A1

9. Coexisting Medical Conditions

10. Results from a self-report survey of vulvodynia patients administered by the National Vulvodynia Association
Disorder
Number surveyed
Have It
Suspect It
Chronic Fatigue
1566
12.6%
19.9%
Endometriosis
1452
15.6%
4.4%
Fibromyalgia
1547
20.0%
15.4%
Interstitial Cystitis
1662
25.2%
22.0%
Irritable Bowel
1675
34.9%
15.8%
Low Back Pain
1729
55.5% -
Migraine Headaches
1564
31.2%
Chemical Sensitivities
1595
27.2%
18.2%
Other Chronic Pain
2150
40.5%

12. Differential Diagnosis and Treatment of Vulvar Pain

13. 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

14. 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)

15. Objective Findings
Turner, M.L.C. and Marinoff, S.C., General principles in the diagnosis and
treatment of vulvar diseases, Dermatologic Clinics, 10 (1992)

16. Vulvar Dermatoses

17. Lichen Simplex Chronicus (LSC)

18. 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

19. 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

20. 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.

21. LSC: Treatment
Remove irritants or allergens (if known) and stop all topicals, soaps, douches, etc.
Sitz baths or compresses 1-2x/day for 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

22. Lichen Sclerosus (LS)

23. 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

24. 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

25. 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.

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

27. 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

28. Lichen Planus (LP)

29. 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

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

31. 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.

32. 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

33. 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

34. 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

35. Redneck Yacht

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

37. 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)

38. 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

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

40. 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)

41. 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). ◄

42. 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

43. Pathophysiology of VVS
Vestibular Nerve Fiber Proliferation in Vulvar Vestibulitis Syndrome
Westrom, L.V. and Willen, R., Obstetrics and Gynecology, 91 (1998)
FINDINGS
Vestibular neural hyperplasia may provide a morphologic explanation of the pain in vulvar vestibulitis syndrome.

44. 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)
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.

45. 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)
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.

46. 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)
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.

47. 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

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

49. 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)

50. DVY: Presentation and Diagnosis
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

51. 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.)

52. 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

53. Resources

54. Associations Books Internet National Vulvodynia Associationor
Services for patients and health care professionals
International Society for the Study of Vulvovaginal Disease or
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