Benign and malignant diseases of the vulva
ObjectivesThe aim of this lecture is to review the diagnosis and management of common vulval dermatoses and benign conditions
Introduction and Background
Symptoms and signs of vulval skin disorders are common and include
pruritus,
pain and,
Changes in skin colour and texture.
About one-fifth of women have significant vulval symptoms
Vulvar skin disorder
Common causes are:
Dermatitis,
Lichen simplex,
Vulval candidiasis,
lichen sclerosus constitutes 25% and,
lichen planus
Vulval skin disorders investigations
Consider testing for thyroid disease, diabetes and sexually transmitted infections if clinically indicated.
Biopsy is required if the woman fails to respond to treatment or there is clinical suspicion of VIN or cancer, or uncertainty of the diagnosis
Women suspected of having lichen sclerosus or lichen planus should be investigated for other autoimmune conditions if there are clinical symptoms or signs.
Serum ferritin should be checked in women with vulval dermatitis
Skin patch test for vulval dermatitis
Lichen sclerosus
DEFINITION
Lichen sclerosus is a destructive inflammatory condition with a predilection for genital skin
AETIOLOGY
is an autoimmune disorder occurring in genetically predisposed individuals. It may be associated with Vitiligo and alopecia areata and myxoedema, type 1 diabetes mellitus and pernicious anemia
PATHOPHYSIOLOGY
The autoimmune attack is by lymphocytes in the upper dermis, and both the dermo-epidermal junction above and the dermis below suffer – there is liquefactive degeneration of the basal cell layer with destruction of melanocytes and stimulation of dermal fibroblasts to produce a vast sheet of homogenized collagen in the upper dermis
INCIDENCE
The peak ages for presentation are childhood and around or after the menopause. The true incidence is unknown, prevalence has been estimated at between 1 in 300 and a 1:1000 of the population.
It is seen in both sexes, at any body site and in all races; but most commonly affects the genital skin of white women
PRESENTATION IN ADULTS
Affected women present with pruritus and only rarely is pain or dyspareunia a prominent complaint. The condition is commonly misdiagnosed as ‘recurrent candidiasis”
The first changes occur on the labia minora and clitoral hood. They become swollen with a rubbery oedema and take on a dull creamy colour.
The whole perineum and genitocrural folds may become bright white with progressive shrinkage and resorption of normal vulval architecture.
Squamous cell carcinoma rarely supervenes (approx. 3–4%); this may begin as a persistent erosion, or hyperkeratotic papule
Treatment
A super-potent topical corticosteroid (e.g. clobetasol propionate ointment) for 6-12 weeks. (dermodin ointment).
LICHEN SIMPLEX CHRONICUS
A vicious circle can be setup in which scratching (for whatever reason) leads to thickened itchy patches of skin. Further scratching worsens the situation and eventually a subconscious scratching habit can become established. It may be triggered by heat (e.g. in bed, after hot bathing), irritants (e.g. soaps, bubble baths, etc.) or psychological factors. The habit is difficult to break, especially because patients are largely unaware of their scratching behavior.
Management
Management involves reassurance and an explanation of the ‘itch-scratch’ cycle. This often heightens at times of stress.
An emollient and short-term grade I or II topical corticosteroids can help break the habit. Behavioral therapy can be particularly successful at helping to extinguish the habit of scratching.
Bartholin’s Cyst and Abscess
Bartholin’s glands are located at 4 & 8 o’clock positions on the posterior-lateral vaginal orifice. Obstruction of the ducts leads to cyst formation or abscess if infected.
No need to treat small asymptomatic cyst. Large cyst may cause pain or dyspareunia. Bartholin’s abscess is diagnosed if severe pain, erythema, and tenderness are present.
Management
Marsupialization or excision
Malignant diseases of the vulva
Vulval cancer is rare accounting for 6% of gynecological malignancies.
Most vulval cancers occur after the menopause with the peak incidence between 65 and 75 years, but the incidence has increased in younger women with 15% cases occurring in women less than 40 years of age.
Risk factors for developing vulval cancer
Lichen sclerosus (4% risk of developing cancer)
Vulval intra epithelial neoplasia (VIN) and multifocal disease
Paget disease
Melanoma, in situ
Smoking
Immunosuppression
Advanced age
History of cervical neoplasia
Histology
The majority of vulvar cancers are squamous in origin
Presentation
Most squamous cancers involve primarily the medial aspects of the labia majora
Diagnosis
Biopsy of the tumor
Presenting symptoms in vulvar carcinoma
Pruritus
Vulvar lump or swelling
Vulvar ulceration
Bleeding
Pain or soreness
Urinary tract symptoms
Discharge
Management of vulvar cancer
Combinations of surgery, radiotherapy and chemo radiation.
Toxic shock syndrome
There is a link between this syndrome and certain organisms found within the vagina of affected women, it is not a vaginal infection.
There are multisystem manifestations and similarities with other conditions produced by staphylococcal toxins.
92% of reported cases were associated with menstruation, and 99% of these were in tampon users
Clinical features
The characteristics of the syndrome are an abrupt onset of pyrexia equal to or greater than 38.9C, myalgia, diffuse skin rash with oedema and blanching erythema, like sunburn, and subsequent(1–2 weeks later) desquamation of the palms and soles.
Less commonly, vomiting and diarrhea symptomatic of hypotension is seen.
Laboratory results
leukocytosis,
thrombocytopenia,
and increased serum bilirubin,
Liver enzymes and creatine phosphokinase. Staphylococcus aureus can be identified frequently from the vagina but blood cultures are usually negative.
Mortality rates from the syndrome were reported initially as high as 15% but fell to 3% by 1981
Treatment is as for any septicemia and includes intravenous fluids and, where necessary, inotropic support. The cause, where possible, should be eliminated and a β lactamase-resistant penicillin given parenterally. Relapse can occur with subsequent menstruation and it is recommended that tampons should not be used until Staphylococcus aureus has been eradicated from the vagina. Relapse has been described in the peuperium
Vaginal cancer
Vaginal cancer is rare and accounts for only 1–2% of all gynecological malignancies. They arise as primary squamous cancers or are the result of extension from the cervix or vulva. A wide age range (18– 95 years) with the peak incidence in the 6th decade of life and a mean age of approximately 60–65 years. There would appear to be no relationship with race or parity.
Etiology
Prior lower genital tract intraepithelial neoplasia and neoplasia (mainly CIN and/or cervical carcinoma)
• HPV infection (Oncogenic subtypes).
Previous gynecological malignancy.
Presentation
Vaginal bleeding. Accounts for more than 50% of presentations.
• Vaginal discharge
• Urinary symptoms
• Abdominal mass or pain
• Asymptomatic 10%.
Pathology
Eighty to ninety percent of tumors are squamous cell carcinoma
The upper third of the vagina is the site most frequently involved
Presentation ranging from small ulcers less than a centimeter in diameter to large pelvic masses, although the majority of tumors are 2–4 cm in maximum diameter.
Treatment
The majority of cases of vaginal carcinoma are treated using pelvic radiotherapy in the form of brachytherapy although surgical excision is an appropriate form of management in selected cases