Disorders of the vulva

Transcription

1 Vulval lesions

2 Disorders of the vulva 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 causing chronic vulval pruritus and dyspareunia. Presentation Can present at any age,but is 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 Uncomplicated lichen sclerosus Whitening of skin in atypical figure of eight appearance

7 Complicated lichen sclerosus Loss of anatomy (burying of the clitoral hood, loss of labia minora, shrinkage of the introitus)

8 Treatment Topical potent corticosteroids( clobetasol propionate ointment/dermovate),skin care and use of emollients. 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

9 Complications, cancer risk and followup Main issues are the scarring complications of the disease. Involves clitoral burying and narrowing of introitus Management involves regular use of the steroids ointment with massage to the fissure Surgery to divide adhesions and postoperative vaginal trainers to prevent scar tissue reforming. Difficult cases should be referred to a vulval service. 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 leaflet).

10 Lichen planus Autoimmune inflammatory skin condition with features similar to lichen sclerosus. Patients usually postmenopausal and 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. The condition exhibits Koebner phenomena when minor degrees of skin trauma leads to development of disease. Treatment with topical, strong corticosteroids and good skin care with emollients. A biopsy confirms diagnosis.

11 Lichen Planus Patients with erosive lichen planus presents with predomnantly vulval pain. Symptoms usually felt within the vagina and urination painful as urine comes into contact with the skin acts as an irritant. The vaginal disease can take appearance of an eroded and glazed appearance which is tender to touch Patients with difficult/unresponsive disease best managed by a specialist vulval service.

12 Lichen planus of the labia majora with multiple welldefined, pink papules on the labia majora

13 Erosive lichen planus of the vulva with an eroded appearance to the inner labia

14 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, is an immediate, non-immunological, local inflammatory reaction, characterised by erythema,following application of chemical substance to cutaneous site Topical corticosteroids with an antibacterial/fungal are of help Patch testing with dermatologist to be considered when attack has settled. Liberal use of emollients to soothe and rehydrate the skin important.

15 Vulval dermatitis with post inflammatory pigmentation of the skin

16 Vulval eczema Eczema is described as 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 and treatment is a combination of emollients and topical corticosteroids. Referral to a vulval dermatologist to be considered if symptoms fail to settle.

17 Vulval Eczema

18 Psoriasis Chronic papulosquamous proliferative inflammatory skin disease in which epidermal cell cycle is reduced leading to 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 and management is with emollients and topical corticosteroids. Coal tar preparations on the vulva to be avoided.

19 Intra epithelial neoplasia

20 VIN 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)

21 Classification Differentiated 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. Usual type VIN 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.

22 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.

23 Raised leucoplakic warty lesion on labia minora

24 Management 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 Key part of management is general care of vulval skin and avoidance of potential irritants that worsen vulval irritation Important to enquire about over-the-counter preparations that aggravate skin conditions. Need to be seen on regular basis for vulvoscopy or careful clinical assessment and biopsy of any suspicious area. Colposcopy examination to exclude intraepithelial neoplasia at other sites. Should be encouraged to perform self-examination to monitor any suspicious areas.

25 Treatment of lesion Simple and radical vulvectomy are inappropriate surgical treatments owing to adverse effects on body image. Local excision is adequate with same recurrence rates and provides specimen for histological diagnosis. If surgical treatment not undertaken,adequate biopsy sampling is required to reduce risk of unrecognised invasion. Complete response rates are higher with excision than with ablative or medical treatment techniques. 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.

26 Thank you!