Def: group of diseases of affecting the vulva & characterized by:
Abnormal growth & maturation of epithelium.
Confined to epithelium i.e. not invade the basal layer.
May involve part or whole thickness of the epithelium.
A- Old classification:
1- Hypertrophic dystrophy: (with or without atypia): squamous cell hyperplasia.
2- Atrophic dystrophy: lichen sclerosis.
3- Mixed dystrophy: (with or without atypia).
N.B.: dystrophy is a disorder of structure or function due to altered nutrition.
B- New classification:
2- Non-neoplastic epithelial lesions: lichen sclerosis, squamous cell hyperplasia & lichen planus.
3- Mixed non –neoplastic & neoplastic epithelial disorders:
4- Vulval intraepithelial neoplasia (VIN): Squamous type. Non-squamous type e.g. Paget’s disease.
5- Invasive malignancy.
Lichen sclerosus (Atrophic dystrophy)
Incidence: the commonest cause of white lesions of vulva.
Aetiology: unknown but may be:
1- Genetic predisposition.
2- Environment factors e.g. chronic irritation.
4- ↑ Chalones: specific tissue proteins secreted by epidermis that normally inhibit mitosis.
Pathology: hyperkeratosis & thin inactive epithelium.
1) Pruritus vulvae is the most common: severe & persistent.
N.B. scratching is initiated by epithelial changes & not the reverse.
2) Contracture of introitus (kraurosis vulvae).
1) The disease doesn’t affect the vagina.
2) Ivory white glistening papules with cellophane-like skin surface.
Investigations: Biopsy is essential to confirm the diagnosis & exclude malignancy.
Def.: inflammatory dermatosis with mucocutaneous eruption.
Aetiology: unknown (may be autoimmune triggered by exogenous antigens e.g. drugs).
Pathology: saw toothed rete ridges.
C/P: depends on the extent of disease:
1) Vaginal discharge & burning.
2) Severe itching scratching scarring dyspareunia.
B. Signs: thick white indurated small plaques anywhere from the clitoris to the anus.
C. Differential Diagnosis
• •• • Lichen planus is most commonly misdiagnosed as lichen sclerosus.
• •• • Both diseases can have white plaques with an intensely inflammatory reaction on the vulva leading to scarring, agglutination, and destruction of the vulvar architecture.
• •• • The hyperkeratotic lesions of LP, however, do not typically exhibit the typical waxy or ‘‘cigarette paper’’ appearance of lichen sclerosus.
• •• • Vaginal involvement is extremely rare in patients with lichen sclerosus.
• •• • Interestingly, lichen sclerosus and lichen planus can coexist in the same patient.
Treatment: (usually difficult)
1- Avoid possible predisposing factors.
2- Treatment of 2ry infection.
3- Local steroid.
Squamous cell hyperplasia
Aetiology: reaction to medical problems e.g. vulvitis.
Pathology: hyperkeratosis & acanthosis.
A.Symptoms: severe pruritus that increases with heat, stress, menses & tight synthetic clothes.
1) Thick skin with ulcers.
2) May be unilateral or bilateral.
3) Localized or affecting the whole vulva.
Investigations: as lichen sclerosis.
1) Stop pruritus.
2) Local steroids: local cream & local intralesional injection.
3) Treatment of associated infection.
B. Surgical: vulvectomy.
N.B. Corticosteroids: cause dermal atrophy & also relieves pruritus.
Precancerous lesions of Vulva
1. Vulval intraepithelial neoplasia:
♦ ♦♦ ♦ VIN I, VIN II and VIN III
♦ ♦♦ ♦ Bowen’s disease and Paget’s disease
♦ ♦♦ ♦ Carcinoma in situ, also called carcinoma simplex.
2. Vulval dystrophy:
♦ ♦♦ ♦ Types with atypia i.e. Hypertrophic and mixed types.
3. Human papilloma virus;
♦ ♦♦ ♦ Serotypes 11, 16, 18.
♦ ♦♦ ♦ Verrucous carcinoma; warty lesion caused by HPV infection
Vulval Intraepithelial Neoplasia (VIN)
1- Carcinoma in situ simplex: malignant cells in all layers of epidermis with no invasion of basement membrane.
2- Bowen’s disease:
Def: intra-epithelial neoplasm in which Bowen’s cells “large multinucleated cells with perinuclear cytoplasmic vaculations’’.
Special characters: 1) Occurs at young age. 2) Long in-situ stage. 3) High incidence of association with cancers of other organs especially cancer cervix.
3- Paget’s disease:
Def: locally malignant tumour presented with eczema-like lesions with ulceration.
Special character: association with adenocarcinoma of sweat glands in 25% of cases.
Pathology: Paget’s cells which are “large cells -round or oval- with large, central hyperchromatic nuclei & perinuclear cytoplasmic vaculations’’.
VIN I: Deep ⅓ of epithelium has mild atypia, dysplasia & abnormal cells.
VIN II: Deep ⅔ of epithelium has mild atypia, dysplasia & abnormal cells.
VIN III: Full thickness has mild atypia, dysplasia & abnormal cells.
C/P: (age around 35 years)
2) Symptomatic: pruritus vulvae.
1) Vulva may be normal.
2) Vulva may be abnormal: multifocal colored patches that may in vulva, perineum & vagina.
1) Biopsy from suspected areas:
It’s the definitive method of diagnosis.
Taken by either colposcopy, Toluidine blue test or tetracycline fluorescence
2) Colposcopy: white lesions & abnormal vasculature appear as evidence of abnormality.
3) Toluidine blue test (Collin’s test).
• Method: 1% Toluidine blue solution is applied to vulva for 2- 4 minutes then, the vulva washed thoroughly with 1 % acetic acid.
• Result: areas remain blue in colour are abnormal areas.
4) Tetracycline fluorescence: foci of increased metabolic activity (malignant cells take the drug).
A. Medical treatment: Topical 5-flourouracil ointment.
• Indication: alternative to vulvectomy in young patients.
• Action: cytotoxic agent that inhibits DNA & RNA synthesis.
B. Surgical treatment:
1) Local excision of affected area with safety margin.
2) Simple vulvectomy: in patients >50 years.
3) Skinning vulvectomy: the bare area after vulvectomy is covered by autogenous skin graft.
4) Laser ablation: using colposcopy.
C. Follow up: is mandatory (recurrence = 30-50%).
Def.: malignant change developed within epithelium lining the vulva.
Incidence: the 4th common invasive genital malignancy (4%).
A. Risk factors (old age > 60 years & conditions with prolonged irritation e.g. vulval dystrophy).
B. Precancerous lesions:
1. VIN: (3 forms & 3 grades).
2. HPV infection (serotypes 11, 16, 18 may predispose to verrucous carcinoma).
3. Vulval dystrophy with atypia.
A. Macroscopic picture:
* Cauliflower mass.
* Nodular lesions. * Malignant ulcer.
B. Microscopic picture:
1. Direct spread: to the surrounding structures.
2. Lymphatic spread:
• Superficial inguinal lymph nodes.
• Superficial femoral lymph nodes.
• Deep inguinal lymph nodes.
• Deep femoral lymph nodes.
• Iliac LN. (ext., int., & common).
• Para-aortic lymph nodes.
3. Blood spread: liver, lung, bone & brain.
4. Implantation or direct contact: kissing ulcers.
D. Complications: hemorrhage is the commonest cause of death.
EPITHELIAL DISORDERS OF THE VULVA
1) Pruritus vulvae is the earliest symptom.
3) Postmenopausal bleeding.
4) Vulval swelling, and soreness.
5) Serosanguinous discharge.
1) General examination: assess general condition & detect distant metastasis.
2) Abdominal examination: palpate liver for metastasis.
3) Local examination: detects macroscopic lesions.
A. For early detection:
1) Cytology (scraping).
2) Colposcopic directed biopsy.
3) Toluidine blue test.
B. Confirm the diagnosis: biopsy.
C. Assess the spread: metastatic work up e.g. (CXR, CT, & MRI).
D. Pre-operative preparation:
* CBC & blood sugar.
*urine analysis, KFT, LFT.
1) Other causes of vulval swellings, vaginal ulcers & pruritus vulvae. 2) Other causes of postmenopausal bleeding.
A. Prophylactic treatment:
avoid risk factors & proper treatment of risk factors.
B. Active treatment:
Plan of treatment:
Stage 0: wide local excision.
Stage I, II: radical vulvectomy & deep femoral lymphadenectomy.
Stage III or IV or recurrent cases: add radiotherapy.
Prognosis: 5 years survival rate is 85% in the absence of inguinal spread.