Vulvitis

Aetiology

Infection

• Candida, trichomoniasis, bacterial vaginosis.
• Pubic lice, threadworm, scabies.
• Herpes simplex, urinary tract infection (UTI), vulval vestibulitis.
• Group A beta-haemolytic streptococcal (GAS) infection has been reported in prepubertal girls and, on rare occasions, in adult women.^[1][2]

Dermatological conditions

• Contact or seborrhoeic dermatitis.
• Psoriasis, lichen simplex/planus/sclerosus.
• Squamous cell hyperplasia.

Neoplasia

• Squamous cell carcinoma (90% of cases have vulvitis).

Atrophic

• Atrophic vaginitis.
• Breast-feeding can result in lowered oestrogen levels and consequent vulval symptoms.

Miscellaneous

• Poor hygiene.
• Pregnancy.
• Generalised pruritus.
• Psychological problems.
• Idiopathic - uncommon, and only diagnosed when all other causes have been excluded.
• Vulvitis circumscripta plasmacellularis (Zoon's vulvitis). This is a distinct entity, presenting as shiny, atrophic, erythematous plaque of the vulva.^[3]

Miscellaneous pain syndromes

• Vulvar vestibulitis syndrome - thought to be due to nonspecific inflammation of the minor vestibular glands.^[4]
• Vulvodynia - causes chronic vulval and pelvic pain, of unknown aetiology.^[5]

Conditions causing the vulva to become excessively moist, such as vaginal discharge and urinary incontinence, lower the defences against commensal organisms and make the area vulnerable to infection.

Epidemiology

Exact figures are not available because the term covers so many different conditions and is likely to be under-reported, as patients may self-treat and not consult a health professional.

• Approximately 10% of women seen by gynaecologists experience pruritus vulvae.^[6]
• Females of all ages are affected, from prepubertal girls to the elderly.^[6][7]
• One community survey of 303 women in the USA reported that a fifth of the sample had experienced lower genital tract discomfort for at least three months, and one in ten women had recurrent symptoms.^[8]

Presentation

History

The presenting symptoms are itch, irritation, soreness, rawness or burning:^[6][8]

• Cyclical pruritus is likely to be due to vulval candidiasis.^[9] Dermatitis may cause intermittent itching, with flares associated with exposure to irritants.
• Vaginal discharge may be a pointer to infection.
• A personal or family history of skin disease (eg atopy, psoriasis, eczema) or autoimmune disease (associated with lichen sclerosus) may be significant.
• Enquiry should be made about general health and any stress factors.

Other information of importance includes current medication, previous treatment (prescribed or purchased), obstetric and gynaecological history (including genital warts) and any potential allergens or sensitiser, such as sanitary wear, soap or detergent.

Examination^[6][9]

The examination should be performed in good lighting to assess subtle changes in the skin. A chaperone should be offered.

• As a minimum, the vulva, pubis and perianal area should be examined. The cervix and vagina should be included if genital infection is suspected.
• Other areas of skin should be examined if there are rashes elsewhere.
• If the presenting complaint is mainly dyspareunia, pelvic muscle tone may need to be assessed.
• If the appearance is essentially normal it may be worth proceeding to see whether the pain is localised and provoked by light touch (suggestive of vulvar vestibular disorder) or is more generalised and not provoked by touch (suggestive of vulvodynia).

Differential diagnosis

Vulvitis is a syndrome which presents primarily as vaginal pruritus or pain. Consideration of differential diagnosis should therefore focus on the cause of either symptom by narrowing down the extensive list of aetiologies (see 'Aetiology', above).

Investigations^[6]

• The clinical diagnosis may be apparent from the history and examination. However, investigations are often required to support the clinician's suspicions. Blood tests may include fasting glucose, FBC, serum ferritin and TFTs..
• If an infection is suspected, appropriate swabs or cultures should be taken to look for conditions such as candida or bacterial vaginosis.
• If a sexually transmitted disease is suspected, appropriate swabs and/or blood tests should be arranged. Such tests can be performed in genitourinary clinics but are increasingly being organised in primary care.
• Skin biopsy may be required in cases of diagnostic difficulty (as a rule of thumb, any skin lesion not responding to a six-week course of treatment).^[9] This is usually performed in secondary care or by a GP with special interest.

Associated diseases

Depending on cause this could include:

• Diabetes.
• Immune deficiency states.
• Urinary incontinence.
• Perimenopause, and postmenopausal oestrogen deficiency.
• Faecal incontinence.
• Any cause of generalised pruritus, eg liver disease, lymphoma.
• Psychological problems.

Management

• Most cases will have an identifiable cause, so accurate diagnosis is an important precursor to management.^[10]
• Potential malignant change in an area of pruritus is always a possibility, so patients with unresolved symptoms should be reviewed regularly.
• Suspected vulval carcinoma needs urgent referral as per local and national guidelines.^[11]
• Consider referring patients with non-suspicious skin changes and negative microbiology for patch testing, as these cases are often allergic in nature.
• Psychological factors can be a cause and a complication of vulvitis, and an holistic approach should be taken.^[6]

General advice

• Avoid contact of the vulval skin with soap, bubble bath, shampoo, perfumes, personal deodorants, wet wipes, detergents, textile dyes, fabric conditioners and sanitary wear.
• Avoid tight-fitting garments that may irritate the area.
• Partners should avoid use of spermicidally-lubricated condoms.
• Patients should be given accurate and clear written information to reinforce these measures.^[12]

Pruritus vulvae of unknown cause

In the absence of a specific diagnosis, or whilst waiting for results, the following treatments can be tried. Most are based on the empirical experience of experts, as there is little published evidence:^[6]

• Emollients can be used as an adjunct to other treatments and are suitable for easing itching in almost all types of vulval disease; they can be used in addition to most other therapies. They can also be used as a soap substitute or moisturiser. There is wide patient variability and lack of comparative evidence, so the choice of preparation can be left to individual preference. If topical steroids are used as well, the emollient should be used first and the steroid 10-20 minutes later. This ensures the skin is moisturised and avoids spread of the steroid to normal skin.
• Sedating oral antihistamines appear to work by promoting sedation rather than blocking the action of histamine. Sedative antidepressants have been used with similar benefit.^[13]
• Low-potency topical corticosteroids, eg hydrocortisone 1% ointment, can be considered as a short trial (1-2 weeks). Potent steroids should be avoided as they can affect surface features and confuse the diagnosis should subsequent specialist referral be required. Specialist referral is indicated if there is no response to steroids.

Specific management (known cause)

This will depend on the underlying condition and the results of investigations. Potent steroids should only be used if the prescriber is confident in the diagnosis. This is usually after confirmation by a specialist, often on the basis of biopsy results.

Infection

Vulval and vaginal infections should be treated with the appropriate antibiotic, antifungal, antiviral or other antimicrobial agent.

Consider investigating and treating the partners of women with recurrent Group A beta-haemolytic streptococcal (GAS) infection. Such men have been found to have a high incidence of GAS in the bowel which is passed on via contamination of bedding. Treating both partners sometimes results in resolution of the condition.^[1]

Dermatological conditions

• Contact dermatitis - this is mainly centred on irritant avoidance, with topical corticosteroid treatment as a secondary measure to relieve itching.
• Seborrhoeic dermatitis and psoriasis - these are usually treated with judicious use of topical corticosteroids (sometimes combined with an antibacterial or anticandidal agent). Ketoconazole shampoo can be used as body wash for seborrhoeic dermatitis.
• Lichen simplex can be treated with topical betamethasone for 1-2 weeks to break the itch-scratch cycle.
• Lichen sclerosus and lichen planus may respond to short-term regular potent or superpotent topical corticosteroids followed by maintenance application.^[14] Women with lichen sclerosus have a small risk (2-5%) of developing carcinoma, so long-term follow-up is recommended.^[15][16] Regular use of a simple moisturiser may lessen attacks and reduce the requirement for steroids.^[17]

Atrophic vaginitis

Declining oestrogen levels may be treated with topical oestrogen therapy or as part of a systemic hormone replacement treatment. The need for long-term topical treatment should be constantly reviewed. Oral progesterone may need to be added to prevent endometrial hyperplasia.

Zoon's vulvitis

This normally responds to high-potency topical steroids.^[18]

Other conditions

In both the conditions below, examination and investigations are usually normal:

• Vulvar vestibular syndrome - this is also known as vestibulitis, vestibular pain syndrome, vestibulodynia and localised vulval dysaesthesia. Altered pain perception is the major feature of this syndrome.^[9] Management is often difficult. A number of treatments have been tried, including Xylocaine® gel, pelvic floor retraining with biofeedback, low-dose tricyclic antidepressants, newer agents for neuropathic pain, and cognitive behavioural therapy. Rarely, vestibulectomy is offered as a last resort.^[19]
• Dysaesthetic vulvodynia - this is also known as essential vulvodynia and generalised vulval dysaesthesia. The predominant symptom is chronic, poorly localised vulval burning or pain. The exact aetiology is unclear, but the condition shares some features with neuropathic pain syndromes. Low-dose tricyclic antidepressants are the standard treatment for dysaesthetic vulvodynia. Gabapentin, imipramine and venlafaxine have also been reported to be beneficial.

When to refer

Referral is indicated if:

• There is an unexplained vulval lump or vulval bleeding due to ulceration.
• Sexually-transmitted infection is suspected, and there is no capacity for the clinician to do screening tests.
• If a dermatological diagnosis is suspected but there is no response to treatment.
• If contact allergy is suspected and patch testing is required.
• If an underlying cause has not been identified and symptoms do not respond to simple advice or a short trial of topical hydrocortisone.

Complications

• Night-time pruritus can lead to sleep loss and reduce quality of life.
• If correctly diagnosed, most underlying causes can be successfully treated.
• Failure to diagnose serious underlying conditions, such as neoplasia, can be fatal.
• Anxiety states and neuroses can lead to psychosexual problems. Some women may find that pruritus is made worse rather than helped by topical products.^[6]

Prognosis

• Misdiagnosis of malignancy can lead to late treatment and significant mortality.
• Most cases of pruritus resolve once the correct diagnosis is made and appropriate treatment instituted.
• Long-term follow-up studies have shown that even chronic pain syndromes tend to resolve eventually.^[20]