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.
Classifications:
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.
3- Autoimmune.
4- ↑ Chalones: specific tissue proteins secreted by epidermis that normally inhibit mitosis.
Pathology: hyperkeratosis & thin inactive epithelium.
C/P:
A. Symptoms:
Asymptomatic.
Symptomatic:
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).
B. Signs:
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.
Treatment:
Lichen planus
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:
A. Symptoms:
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
(Hypertrophic dystrophy)
Aetiology: reaction to medical problems e.g. vulvitis.
Pathology: hyperkeratosis & acanthosis.
C/P:
A.Symptoms: severe pruritus that increases with heat, stress, menses & tight synthetic clothes.
B. Signs:
1) Thick skin with ulcers.
2) May be unilateral or bilateral.
3) Localized or affecting the whole vulva.
Investigations: as lichen sclerosis.
Treatment:
A. Medical:
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)
Pathology:
Forms
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’’.
Grading
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)
Symptoms:
1) Asymptomatic.
2) Symptomatic: pruritus vulvae.
Signs:
1) Vulva may be normal.
2) Vulva may be abnormal: multifocal colored patches that may in vulva, perineum & vagina.
Investigations:
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).
Treatment:
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%).
VULVAL CARCINOMA
Def.: malignant change developed within epithelium lining the vulva.
Incidence: the 4th common invasive genital malignancy (4%).
Aetiology:
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.
Pathology:
A. Macroscopic picture:
* Cauliflower mass.
* Nodular lesions. * Malignant ulcer.
B. Microscopic picture:
*SCC (90%).
*BCC. *Adenocarcinoma.
C. Spread:
1. Direct spread: to the surrounding structures.
2. Lymphatic spread:
1ry group:
• Superficial inguinal lymph nodes.
• Superficial femoral lymph nodes.
2ry group:
• Deep inguinal lymph nodes.
• Deep femoral lymph nodes.
3ry group:
• 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
C/P:
A. Symptoms:
1) Pruritus vulvae is the earliest symptom.
2) Pain.
3) Postmenopausal bleeding.
4) Vulval swelling, and soreness.
5) Serosanguinous discharge.
B. Signs:
1) General examination: assess general condition & detect distant metastasis.
2) Abdominal examination: palpate liver for metastasis.
3) Local examination: detects macroscopic lesions.
Investigations:
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.
*ECG.
D.D.:
1) Other causes of vulval swellings, vaginal ulcers & pruritus vulvae. 2) Other causes of postmenopausal bleeding.
Treatment:
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.