1. Dr U S SABITHA Assistant Professor, Dept of Obgyn, PESIMSR
VULVAL DISORDERS
Dr U S SABITHA
Assistant Professor,
Dept of Obgyn, PESIMSR

2. Disorders of the Vulva Classification
Terminology standardised by the International Society for the Study of Vulvovaginal Disease(ISSVD)
Classification
1. Nonneoplastic epithelial disorders of vulva
• Lichen sclerosus
• Squamous cell hyperplasia (SCH)
• Other dermatoses (e.g. psoriasis, lichen planus)
2. Intraepithelial neoplasia
– Squamous vulvar intraepithelial neoplasia (VIN)
– Non-squamous intraepithelial neoplasia
• Extramammary Paget's disease
3. Invasive disease (vulval cancer)

3. NON NEOPLASTIC LESIONS

4. LICHEN SCLEROSUS
Inflammatory dermatosis that predominantly affects anogenital area Presentation: chronic vulval pruritus and dyspareunia. more commonly seen in postmenopausal women Etiology Autoimmune disease Associated with other autoimmune diseases among patients (40%)

5. History and Clinical Findings
• Commonly present with vulval and perianal itching
• Pain may occur, but is usually secondary to skin trauma from scratching.
• Fissures and erosions might occur from local trauma.
• Dysuria as urine comes into contact with the split skin
• Narrowing of introitus from the scarring effect causing
dyspareunia
• Skin often atrophic, classically demonstrating subepithelial haemorrhages (ecchymoses),and may split easily.
• Continuing inflammation results in inflammatory adhesions.

6. Lichen sclerosus Histology
Epidermal atrophy, dermal edema, hyalinization of collagen and sub dermal chronic inflammatory cell infiltrate
Epidermal hyperkeratosis with elongation of rete pegs

7. Uncomplicated Lichen Sclerosus
Whitening of skin in atypical figure of eight appearance

8. Complicated Lichen Sclerosis
Loss of Anatomy (burying of the clitoral hood, loss of labia minora, shrinkage of the introitus)

9. Treatment
• Topical potent corticosteroids (clobetasol propionate ointment/Dermovate), skin care and use of emollients – 54-96% have resolution of the symptoms • Dermovate massaged into area of fissure and vaginal dilators suggested to help overcome introital narrowing and treat vaginismus • Approximately 4–10% of women will be resistant to steroids • Recommended second-line treatment is topical tacrolimus under supervision of a specialist clinic. • Use for longer than 2 years not recommended owing to risk of potential malignant transformation. • Surgery and CO2 laser vaporisation have role in restoring function impaired by agglutination and adhesions such as urinary retention or narrowing of the vaginal introitus

10. Complications, Cancer risk and Followup
• Main issues are the scarring complications of the disease. • Involves clitoral burying and narrowing of introitus • Increased risk of squamous cell cancer of vulva in women with lichen sclerosus (2-4%). • Follow up important • Patient education and self-examination to detect potentially an early cancer (self-examination).

11. Lichen planus • Autoimmune inflammatory skin condition
• Usually middle aged woman (30-60 years)
present with vulval itching and/or pain.
There are two clinical variants:
• In classical type, skin lesions are isolated polygonal ,flat topped white pearly papules on skin of vulva.
• exhibits Koebner phenomena
• A biopsy confirms diagnosis.
• Treatment with topical, strong corticosteroids and emollients.

12. Lichen Planus Patients with erosive lichen planus
- Predominantly vulval pain.
- urination painful as urine comes into contact with the skin
- vagina- an eroded and glazed appearance- tender to touch
Histology
Liquefactive degeneration of basal epidermala layer,long &pointed rete edges with parakeratosis and acanthosis and dense dermal infiltrate of lymphocytes close to dermaepidermal margin

13. Lichen Planus
Lichen planus of the labia majora with multiple welldefined, pink papules on the labia majora

14. Lichen Planus
Erosive lichen planus of the vulva with an eroded appearance to the inner labia

15. Contact Dermatitis
• Two types of contact dermatitis: allergic and irritant.
• Allergic dermatitis has an immunological basis and is the classic delayed-type hypersensitivity reaction .
- Removal of offending allergen causes dramatic improvement in vulval symptoms.
• Irritant reaction - immediate, non-immunological, local inflammatory reaction, characterised by erythema on contact with chemical substance to cutaneous site
• Topical corticosteroids with an antibacterial/fungal are of help
• Emollients to soothe and rehydrate the skin.
• Patch testing for diagnosis

16. Common contact allergens in vulval dermatitis
Type of allergen Examples
Medicament
local anaesthetics, e.g. benzocaine, dibucaine
Topical steroids, e.g. hydrocortisone
Neomycin, other antibiotics
Imidazoles, e.g. clotrimazole
Preservatives & stabilizers in creams
Parabens, ethylenediamine, clioquinol
Perfumes Balsam of Peru, other fragrances
Clothing dyes Paraphenylene diamine
Metals Nickel

17. Contact Dermatitis
Vulval dermatitis with post inflammatory pigmentation of the skin

18. Vulval Eczema • itchy inflamed skin.
• Even in presence of widespread eczema on the body, vulval symptoms uncommon.
• In those patients affected, vulval lesions include skin erythema, skin scaling and fissuring.
• Usually affects labia and natal cleft.
• Biopsy ? - not usually necessary
Treatment is a combination of emollients and topical corticosteroids.
• Referral to dermatologist to be considered if symptoms fail to settle.

19. Vulval Eczema

20. Psoriasis
• Chronic papulosquamous proliferative inflammatory skin disease in which epidermal cell cycle is reduced
pruritus and soreness.
• Usually evident at other areas on body such as the flexural sites
• The lesion is erythematous,beefy red with well-defined edge.
• With vulval psoriasis, skin scaling not present as the area is moist.
• Biopsy confirms diagnosis
Management is with emollients and topical corticosteroids.
• Coal tar preparations on the vulva to be avoided.

21. Psoriasis

22. VULVAL INTRAEPITHELIAL NEOPLASIA

23. Vulval Intraepithelial Neoplasia
• Denotes intraepithelial lesion of the vulva that shows dysplasia with varying degrees of atypia. • The lesion is not invasive but has invasive potential. • The current classification for VIN by International Society for the Study of Vulvar Diseases (ISSVD): -Differentiated VIN (VIN simplex) -Usual type (VIN basaloid, warty and mixed)

24. Classification Differentiated VIN Usual type VIN
• Unifocal and unicentric • Not graded • High risk of developing squamous cell carcinoma • Postmenopausal women • Associated with lichen sclerosis and usually have a nonviral etiology • Not classically associated with CIN.
• Multifocal and multicentric • Graded the same as CIN • Low risk of developing squamous cell carcinoma • Premenopausal women • Associated with HPV, smoking and immunodeficiency • May have similar pathophysiology to CIN.

25. Vulval Intraepithelial Neoplasia
Clinical Manifestations • Pruritus • Dyspareunia • Lesions (may be raised, erythematous, leukoplakic, keratotic, ulcerated or pigmented in appearance) • Acetowhite change on vulvoscopy. • Approximately 50% of cases asymptomatic. • Represent a field change and, therefore, the cervix and perianal area to be examined to exclude CIN and anal intraepithelial neoplasia.

26. Raised leucoplakic warty lesion on labia minora

27. VIN

28. Management of VIN
• Co existing squamous cell carcinoma of the vulva found in 12–17% of patients.
• Progression to vulvar cancer can happen in 40-60% of untreated VIN and in 4% of treated VIN
• Important to enquire about over-the-counter preparations that aggravate skin conditions.
• Key part of management
- general care of vulval skin and
- avoidance of potential irritants that worsen vulval irritation
- regular check up for vulvoscopy or careful clinical assessment
- biopsy of any suspicious area.
- Colposcopy examination to exclude intraepithelial neoplasia at other sites.
- self-examination.

29. Treatment of lesion
• Simple and radical vulvectomy? - inappropriate owing to adverse effects on body image. • Local excision is adequate with - same recurrence rates - provides specimen for histological diagnosis. • If surgical treatment not undertaken,adequate biopsy sampling is required to reduce risk of unrecognised invasion. Excision or ablation? • Complete response rates are higher with excision than with ablation • The risk of recurrence? - is lower with free surgical margins - However, even with uninvolved surgical margins,there is still a residual risk of recurrence. • Following excision of small lesions primary closure is done, larger lesions however require reconstructive surgeries.

30. PAGETS DISEASE seen in postmenopausal women.
Symptom is mainly pruritus.
On examination, lesions have a florid eczematous appearance with lichenification, erythema and excoriation.
can be associated with an underlying adenocarcinoma.
The gastrointestinal, urinary tracts and the breasts should be checked.
Surgical excision recommended to exclude adenocarcinoma of a skin appendage.
Photodynamic therapy and topical imiquimod have been used with some success.

31. PAGETS DISEASE

32. Behcet syndrome recurrent oral and genital ulcers
ulcers can involve cervix,vulva or vagina.
usually painful
Can leave scar
Treatment :
to control flare-ups and reduce symptoms
- Topical or systemic immunosuppressants.

33. Vulval candidiasis
Irritation and soreness of the vulva and anus rather than discharge.
Diabetes, obesity and antibiotic use may be contributory.
may become chronic
a leading edge of inflammation with satellite lesions extending out from the labia majora to the inner thighs or mons pubis.
Prolonged topical or oral antifungal therapy may be necessary to clear a skin infection

34. What is the most effective model for care provision for the investigation and management of women with vulval skin disorders?
Following women should be seen at specialist clinic:
Complex or rare vulval skin
difficulty with symptom control
who require frequent or prolonged use of ultrapotent topical steroids.
They require biopsy of any suspicious or resistant areas.
Women treated for VIN should be seen on an annual basis at least for inspection of vulval skin and receive information regarding signs and symptoms (such as pain or ulceration) that would prompt an earlier review

35. Summary Disorder Features Management Lichen simplex chronicus
Thickened discolored epithelium due to chronic scratch, leathery appearance
Steroid cream
Lichen sclerosus
Symmetric thinning of labial skin, cigareete or parchment skin, anatomy distorted/adhesions
High potent steroids
Lichen planus
Violaceous papule, whickham striae, lesions elsewhere
Vulval eczema
White or red patches, hyperkeartotic changes
Vulvar psoriasis
Discrete red papules, scaling may be there, lesions elsewhere

36. CASE 1
A 53 year old lady presents with severe pruritis and thinning of the vulval skin, which bleeds on scratching. There is pain, dicomfort and dypareunia and adhesion of labial margins on examination. She also suffers from diabetes.
Histology showed marked epidermal thinning with loss of rete ridges and vacuolation of basal cells

37. CASE 2
A 33 year old lady presents with recent onset of irritation and soreness of the vulva but with a long standing history of vaginal discharge. There is also evidence of an inflammed area on the inside of the thigh.

38. CASE 3
A 42 year old woman presents with recurrent oral and genital ulcers which are very painful. There is extensive evidence of scarring from the previous lesions

39. CASE 4
Significant pruritis in a 52 year old smoker with red plaques and some warty lesions.

40. CASE 5
A 32 year old lady presents with vulval lesions that are smooth and descrete, red in color, but not affecting the vaginal mucosa. The patient also complains of discrete lesions in the flexural areas of the body

41. CASE 6
A 34 year old lady came with complains of vulval itch, clinical exam. showed a white patterened area which is elevated and thickened. Right lower leg had papules with overlying white lacy pattern Hpe showed liquefactive degenertion of the basal epidermal layer, long and pointed rete ridges, acanthosis, dense dermal infitrate of lymphocyte

42. CASE 7
A 56 year lady with eczematoid lesion over the vulva, Hpe- atypical cells with oval nuclei and pale cytoplasm singly or within clusters among the basal cells of the dermis.

43. Plasma Cell Vulvitis or zoon’s vulvitis
postmenopausal women.
pruritus,burning,dyspareunia and dysuria
diagnosed histologically and is characterised by dermal infiltration with plasma cells, vessel dilatation and haemosiderrin deposition.
Etiology ? autoimmune disorder.
Treatment - topical ultrapotent steroids