Vulval Abnormalities and their Management

Within the vulva there is skin, mucous membrane and glands. There is enormous potential for pathology, including psychosomatic problems. Vaginismus and sexual dysfunction in women is covered elsewhere. The scope is so wide that this article aims to give just a general overview with frequent links to other articles that cover aspects in greater detail.

Consider infective causes, dermatological conditions or malignant or pre-malignant change. The likely spectrum of disease varies considerably according to the age of the patient.

Symptoms

Note first the age of the patient. Get as much history as possible before examination:

• Are symptoms sudden or gradual in onset?
• Is there pain, discomfort, irritation, itching or just "something been found"? Dyspareunia has its own article.
• Is there any discharge?
• Distinguish between a vulval and vaginal problem. Many women are not very good at accurate localisation of that part of their anatomy.
• Is there anything to see? Some women will have made close inspection with a hand mirror whilst others would not contemplate looking down there.
• Is there dysuria? If so is there urinary frequency too? There may be no UTI but it is painful to pass urine through an inflamed area.
• If she is sexually active, does her partner have any problems?
• Are there any other current problems such as psoriasis, chickenpox or recent use of antibiotics?

Signs

Signs are as variable as the differential diagnosis and so they will be considered together. Generally, examination will be limited to inspection with gentle parting of the labia. Vaginal examination is often unnecessary and with the current condition it may be too painful to be reasonable.

Some conditions can be diagnosed simply on inspection but others may require swabs and possibly viral culture or even biopsy to confirm the nature.

Congenital Anomalies

These are likely to present around birth and may be noticed by the doctor who performs the examination after birth. The genital tract differentiates between about the 12th and 16th week of gestation. It develops as female unless the influence of testosterone causes male development:

• An intersex state may develop as a result of inadequate hormonal influence in an XY child or as a result of inappropriate testosterone influence in an XX child.
  • Karyotyping may be required to decide which is the case.
• Virilisation may include labial fusion and enlargement of the clitoris.
  • This may be the result of exposure to androgens in utero or as a result of abnormalities of steroid synthesis due to an enzyme abnormality.
  • Of these, the best known is congenital adrenal hyperplasia.
• Labial adhesions tend to present in little girls, but not in the neonatal period.
  • They may be mistaken for congenital fusion of the labia.
  • Labial adhesions are usually asymptomatic, but if the introitus is completely sealed, vaginal micturition with consequent dribbling will occur and the urinary stasis will predispose to infection.
  • Treatment is 1% oestrogen cream, applied 3 times daily for 2 weeks.
  • It is not usually completely effective but at the end of the course it is possible to use a finger or probe to break down the adhesions.
  • If adhesions are dense, some sedation may be required.
  • After the procedure, topical oestrogen or antibiotic cream is important to avoid recurrence of the adhesions.

Nappy rash

• In a small child there may be nappy rash.
  • Urine is an irritant, but if there is faecal contamination the Proteus spp. will convert urea to ammonia.
  • This makes it far more irritant and causes an ammoniacal dermatitis.
• The vulva may be bright red and there may be little patches away from the main part suggesting candida infection too.
• Does the child look cared for or neglected?
• Advise about regular nappy changing and even leaving the child without a nappy for as long as possible.
• Topical hydrocortisone 1% will reduce inflammation. Ointment stays in damp areas better than cream.
• If there is suspicion of candida, a combination of hydrocortisone and an antifungal is required.

Varicella

Little girls with chickenpox sometimes get vesicles around the vulva. This is very painful. Petroleum jelly (Vaseline®) applied 4 times a day helps.

Candidiasis

Candida infection can occur at all ages.

• It may cause vaginitis and a white, curdy discharge as well as vulvitis.
• There is usually pruritus and a red rash. If in doubt, samples can be sent for microbiology but this is usually unnecessary.
• Recent use of a broad spectrum antibiotic increases the risk.
• If the infection is profuse, check for diabetes.
• Topical antifungal creams are usually adequate. Vaginal infection may need treating too.
• If there is a sexual partner, he will require treatment to prevent re-infection.

Genital herpes

Genital herpes is usually caused by the herpes simplex virus (HSV) II and it is spread by sexual contact. The HSV I virus is associated with cold sores on the mouth but oro-genital sex can cause a reversal of this pattern. There are painful, fluid-filled vesicles around the genital area. Virus can be cultured from the fluid.

• The area is so tender that dysuria can even lead to retention of urine.
• All sexual contacts must be notified to seek advice. Referral to a genito-urinary medicine (GUM) clinic may help with diagnosis, treatment and contact tracing.
• The vesicles leave tender ulcers that may take 2 to 4 weeks to heal the first time they occur.
• Typically, another outbreak can appear weeks or months after the first, but it almost always is less severe and shorter than the first outbreak. Although the infection can lie dormant indefinitely, the number of outbreaks tends to decrease over a period of years.
• Management involves salt baths, analgesics, loose underwear, abstention from sexual intercourse until all ulcers are healed and anti-viral therapy.
• The precise dose of anti-viral drug varies between preparations but for famcilovir it is:
  • For the first episode, 250 mg 3 times daily for 5 days.
  • For recurrent infection, 125 mg twice daily for 5 days (in immunocompromised patients, all episodes, 500 mg twice daily for 7 days).
  • Suppression therapy can be used at 250 mg twice daily (in HIV patients, 500 mg twice daily) interrupted every 6 to 12 months.
• The dose of aciclovir for the treatment of herpes simplex is:
  • 200 mg (400 mg in the immunocompromised or if absorption is impaired) 5 times daily, usually for 5 days. Or a child under 2 years use half adult dose and over 2 years the full adult dose.
  • Dose for prevention of recurrence is 200mg 4 times daily or 400 mg twice daily possibly reduced to 200 mg 2 or 3 times daily and interrupted every 6 to 12 months.
  • Herpes simplex, prophylaxis in the immunocompromised is 200 or 400 mg 4 times daily. Children under 2 get half the dose and over 2 years, the full dose.
• Long term treatment is of value if there is frequent recurrence.¹

Genital warts

Genital warts are caused by human papilloma virus (HPV), usually types 6 to 11. Most infections are subclinical but they can cause abnormalities of cervical smears and may well be pre-malignant.² Other HPV types (16, 18, 31, 33 and 35) are less common in visible warts but are strongly associated with squamous cell carcinoma of vulva or penis.³

• Transmission is usually but not invariably sexual.
• Warts usually appear 3 to 6 months after infection.
• There may be pain, bleeding and pruritus.
• Podophyllin paint compound BP destroys the affected skin cells so the warts shrink or disappear. It must be applied accurately and should not be used in pregnancy.
• Other treatments include cryotherapy and electrocautery.
• Genital warts are also called condyloma acuminata. They may be similar to condyloma lata of syphilis and serology may be required to distinguish the two.

Shingles

Herpes zoster does not often affect the genital region but the lesions are characteristic. The anterior two third of the labia majora is innervated from L1 and the posterior third from S2,3,4.

Infestations

Infestations such as scabies and pubic lice may affect the area. Treatment is with an insecticide such as malathion. Up to 20% may also have another sexually acquired disease.

Almost any skin disorder may also affect the vulva but a few are of special note:

• Lichen planus has a very unpleasant variation that causes painful erosive vulvitis.
  • It usually affects older women and the vestibular area and lower vaginal skin can be involved.
  • There is intense erythema, oedema and superficial ulceration.
  • It leads to scarring and introital narrowing resembling chronic lichen sclerosus.
  • Biopsy will distinguish it from other ulcerative disorders including pemphigus, pemphigoid and erythema multiforme.
  • Systemic steroids are often required.
• Psoriasis is not usually itchy, but it can be on the vulva.
  • It does not affect the vaginal mucosa.
  • It may be necessary to take scrapings to exclude tinea infection.
  • There will usually be evidence of the disease elsewhere.
  • Treatment is as for psoriasis elsewhere.
• Behcet's syndrome is a disease of unknown aetiology.
  • It is characterised by recurrent aphthous ulcers, possibly ulcers of the vulva.
  • These usually occur on a cyclical basis and often related to the menstrual cycle.
  • They are sometimes associated with arthritis, usually of the knees.

Swelling or oedema of the vulva can be due to venous or lymphatic obstruction:

• Secondary to malignancy in the pelvis
• Dependent oedema with prolonged sitting in bed
• Pregnancy, where varicosities may appear - usually resolving at the end of the pregnancy
• Haematoma suggests trauma (may have been of a sexual nature)

The modern terms for the conditions previously called the vulvar dystrophies, are lichen sclerosus and squamous cell hyperplasia. Lichen sclerosus often occurs with squamous cell hyperplasia. The terms vulvar dystrophy, kraurosis vulvae, leukoplakia and lichen sclerosus et atrophicus should no longer be used.

• Lichen sclerosus is uncommon:⁴
  • Most cases are in postmenopausal women, although it can occur in prepubescent girls and young women.⁵
  • It can be familial and may affect the male prepuce too.
  • 21% of patients have autoimmune disease, usually a thyroid disorder.
  • 22% have a family history and 44% have various autoantibodies.
  • Lichen sclerosus and squamous cell hyperplasia present with pruritus vulvae, vulvodynia, superficial dyspareunia, or visible lesions.
  • Lichen sclerosus has an appearance called "cigarette paper" skin as it is thin, white, and crinkly. The introitus may shrink with fusion of the labia minora.
  • Biopsy, under local anaesthetic, may need to be repeated from time to time to exclude malignant change.
  • Investigate for other potential autoimmune conditions e.g. thyroid disease.
  • Treat with clobetasol propionate 0.05% ointment. Daily use for one month, alternate days for one month, twice weekly for one month with review at 2-3 months. There is no evidence on the optimal regimen.⁴
  • Long-term maintenance with small amounts usually satisfactory - 30 grams should last 3 months.
  • Oestrogen or testosterone creams should not be used to treat lichen sclerosus.⁶
  • Testosterone is no better than petroleum jelly and there may be adverse effects.⁷
  • It must not be used in children.
  • Sometimes excision of hyperplastic or fissured areas of lichen sclerosus is required if there is no response to therapy.
  • Recurrence rates after excision are high.
  • Lichen sclerosus may even recur in grafted skin.
  • Oral retinoids may be effective in severe recalcitrant disease, but should only be given by a dermatologist, experienced in the use of these agents.⁸
• Squamous cell hyperplasia is largely a disease of older women and is uncommon:
  • There is a nonspecific appearance with thickened, asymmetrical areas and it is white or grey.
  • Lichen sclerosus with squamous hyperplasia is treated as for lichen sclerosus.

Prognosis

• Lichen sclerosus usually responds to therapy in a month or less. Long-term follow-up care is necessary.
• Squamous hyperplasia usually responds to adequate therapy within 2 to 3 weeks.
• Chronic vulvar dystrophy may develop into carcinoma. Most authors agree that lichen sclerosus does not often develop into carcinoma, but it is the most frequent precursor of leukoplakia, which is a risk. Any chronic vulvar irritation may increase the risk of cancer and must therefore be carefully monitored by multiple biopsies to be examined at regular intervals.⁹

Vulvar cancer represents about 5% of all female genital malignancies:

• It is 2 or 3 times more frequent in developing countries.
• Except for rare sarcomas, this cancer is most frequent in women aged 65 to 75 years or older.
• It can occur in younger women.
• They tend to have early microcarcinomas with diffuse intraepithelial neoplasia of the vulva.
• Delay in diagnosis is mainly because the patient does not seek medical attention for many months or because the lesion is treated medically for months, without biopsy for definitive diagnosis.

Squamous cell carcinoma

Squamous cell carcinoma represents about 95% of carcinoma of the vulva:

• Small, warty, or cauliflower-like growths may arise and be confused with condyloma acuminata.
• Squamous carcinoma can appear with atrophic changes, especially lichen sclerosis with hypertrophic epithelium.
• Long-term pruritus, lumps, or masses on the vulva are present in most patients with invasive vulvar cancer. Biopsy and staging precede treatment.
• A small primary lesion of less than 2 cm with superficial invasion can be treated with wide local excision. Larger lesions require more substantial surgery and lymph node biopsy, perhaps with excision.
• Pelvic lymph nodes may be treated by irradiation.
• Surgery tends to be less radical than in former years.

Melanoma

Melanoma is the 2nd commonest malignancy of the vulva at about 5%:

• In this situation it is not usually associated with excessive exposure to sunlight.
• They are suspicious if blue-black in colour with an irregular edge or ulcerated but they may be amelanotic.
• It represents 3% of all melanomas with an incidence of 1 in a million women.
• They are usually over 70.

Paget's disease of the vulva

Paget's disease of the vulva is carcinoma-in-situ:

• It is rare and usually presents in the 7th decade.
• Symptoms are usually pruritus and tenderness or noting a lesion.
• The patient often waits several years before seeking medical advice.
• The extent of the lesion is very variable.
• It is usually squamous cell carcinoma but sometimes an adenocarcinoma.
• There is an association with adenocarcinoma elsewhere (<15%) so investigation
  for other tumours would be advised.⁴
• Wide local excision is adequate if there is no invasion.
• Otherwise, wide margins are necessary to remove the lesion.
• Regular follow up is required as there is a significant risk of recurrence.

• Lichen simplex
• Atopic eczema
• Seborrhoeic dermatitis
• Irritant contact dermatitis (from wetness, incontinence, vigorous cleansing)
• Allergic contact dermatitis (most often due to perfumes or rubber)
• Hidradenitis suppurativa
• Tinea cruris
• Intertrigo
• Molluscum contagiosum
• Pubic lice or scabies
• Syphilis or other sexually transmitted diseases including lymphogranuloma venereum, chancroid or granuloma inguinale.

Sexual abuse can occur at any age (including the elderly) but the problem is particularly well documented in children.

If a child presents with a condition that is usually sexually transmitted (such as genital warts or herpes simplex), sexual activity needs to be considered, but it is not the only cause. Evidence of trauma, especially with a spurious explanation is also suggestive. Other features may include behavioural disorders and inappropriate sexuality of behaviour.

The possibility of sexual abuse taxes the skills of the doctor considerably:

• On the one hand no one would wish child abuse to go undetected and hence to continue.
• On the other hand a false allegation may have devastating consequences, even if later dismissed, and the doctor will be blamed for having created the havoc.

When mentioning the suspicion of sexual abuse to parents there must be abundant expressions of uncertainty and reassurance that this is something that may not be happening, but it is essential to be sure.

The diagnosis of child sexual abuse must be left for the experts.

If a non-expert expresses an opinion and is cross-examined in court his professional credibility will be destroyed:
Every locality should have a named expert whom the child should see, preferably in the next 24 hours.
Be wary about questioning the child too deeply and even more wary about physical examination, as the response of the child is crucial to the expert and previous questioning or physical examination may change this.

The enthusiastic amateur may "trample over the evidence" and make eventual diagnosis and conviction of the offender less likely.

The range of normal appearance of the vulva and vagina in childhood is immensely variable. This is a very important area where failure to confirm abuse where it is happening or false allegations can have a devastating effect on people's lives.

The procedure that used to be known as female circumcision has been widely practiced throughout Africa and the Middle East. The term is inappropriate as it is not simply removal of the clitoral prepuce but a rather gross procedure often involving clitoridectomy and with no degree of precision or skill. The term female genital mutilation (FGM) is more appropriate and is now in common use:

• It is estimated that approximately 138 million women have undergone FGM worldwide with a further 2 million girls a year estimated to be at risk of the practice,¹⁰ mostly in Ethiopia, Somalia and the Sudan.
• In these countries prevalence rates can be as high as 98%.
• In Nigeria, Kenya, Togo and Senegal, the prevalence rates vary between 20 and 50%.
• It is practised more by specific ethnic groups, than by a whole countries.
• Other parts where it is practiced include Yemen, Oman, Iraqi Kurdistan, Ethiopian Jews, and Bohra Muslim populations in parts of India and Pakistan, as well as Muslim populations in Malaysia and Indonesia.

It is usually carried out between the ages of 4 and 10, but it may be performed at any time from birth to delivery of the first baby. The procedure is traditionally carried out by an older woman with no medical training. Anaesthetics and antiseptics are not generally used and the practice is usually carried out using basic tools such as knives, scissors, scalpels, pieces of glass and razor blades. Often iodine or a mixture of herbs is placed on the wound to tighten the vagina and stop the bleeding. As well as the immediate risk, long term complications may include:

• Extensive damage of the external reproductive system
• Uterus, vaginal and pelvic infections
• Cysts and neuromas
• Increased risk of vesico-vaginal fistula
• Complications in pregnancy and child birth
• Psychological damage
• Sexual dysfunction
• Difficulties in menstruation

Knowledge of FGM is important because doctors in this country may have to treat women who have been mutilated in this way.

The practice has been roundly condemned by the WHO, but it is still perpetrated in the original countries and may be sought when these populations emigrate to the developed world. An Act of Parliament of 2003 that came into force in 2004 makes it an offence not just to perform such an operation in the UK but, for the first time, for UK nationals or permanent UK residents to carry out FGM abroad, or to aid, abet, counsel or procure the carrying out of FGM abroad, even in countries where the practice is legal. A similar Act was passed for Scotland in 2005.