Chapter 107: Vulvar disorders

The dermatoses are the predominant cause of vulvar problems and this chapter focuses mainly on the important female genital skin conditions.

The vulva is that part of the female external genitalia lying posterior to the mons pubis, comprising the labia majora, labia minora, clitoris, vestibule of the vagina, vaginal opening and bulbs of the vestibule.¹

The vaginal vestibule is an almond-shaped opening between the lines of attachment of the labia minora. The clitoris marks the superior angle and the fourchette the inferior boundary. It is approximately 4–5 cm long and 2 cm in width.¹ The four main structures that open into the vestibule are the urethra, vagina and the two secretory ducts of Bartholin’s glands. The surface is composed of delicate, stratified squamous epithelium.

The genital area is affected by dermatoses found elsewhere on the skin but management is rendered more complex by the sensitivity and thinness of the skin, a tendency to superinfection, in addition to the psychological problems, including the often-resultant dyspareunia.

The vulval area, which is innervated by nerves arising from L1–2 and S2–4 nerve roots, is sensitive to noxious stimuli but the vagina is not sensitive to pain.² Topical creams, soaps, perfumes and other toilet products irritate the vulva easily—it is an area prone to contact dermatitis.

Clinical manifestations of vulvar disorders include itching, pain, irritation, white mucosal patches, lichenification, erosions and intertrigo³ (see the diagnostic strategy model presented in TABLE 107.1).

Dermatitis

As expected, the various common forms of dermatitis collectively represent the prime cause of a pruritic irritating vulvar skin disorder. They classically present with itching, burning and soreness initiated by scratching leading to white plaques of lichen simplex chronicus. The manifestations can vary from symptoms without a rash to a poorly defined rash without the above symptoms.

The causes of vulvar dermatitis are:

• atopic dermatitis

• irritant contact dermatitis

• allergic contact dermatitis

• seborrhoeic dermatitis

• corticosteroid-induced dermatitis

• psoriasis

Principles of management⁴

• Take an appropriate history, including atopy, skin diseases.

• Check allergens and irritants (e.g. panty liners, soap, bubble bath, perfumed toilet paper, douches, perfumes, condoms, tea-tree oil).

• Check for heat and friction (e.g. synthetic or tight underwear, tight denim jeans, sporting costumes/tights, sweating, vigorous activity—bicycle riding).

• Check gynae-urological history (e.g. oestrogen status, faecal or urinary incontinence, vaginal discharge, ‘thrush’).

• Check psychosexual history (e.g. dyspareunia, partnership issues, depression).

• Carefully inspect the vulva plus the rest of the skin, scalp and nails. Look for lichenification (see TABLE 107.2).

• Appropriate investigations: vaginal swab (? Candida albicans), cervical screening test if due, perhaps patch testing and vulval biopsy for a rare, premalignant or suspected malignant condition, especially if thickening or textural change.

Treatment^3,4

• Provide supportive education and counselling.

• Correct underlying factors (e.g. tight clothes, incontinence, anal discharge, overused topical medications and cosmetics).

• Treat any secondary infection.

• Use aqueous cream moisturiser as cleanser.

• Start with potent topical corticosteroid (e.g. methylprednisolone aceponate 0.1% ointment topically until symptoms resolve) and follow with 1% hydrocortisone to prevent recurrence. Longer treatments such as 2—4 weeks are often required. Review treatment after 2 weeks.⁵

Psoriasis⁵

Psoriasis can affect the genital or perianal area (especially the natal cleft) and appears as a glazed, beefy red plaque without the classic scale seen elsewhere. There may be minimal or no sign of psoriasis on the skin of the body.

The main symptom is itching. It is usual to take swabs to rule out infection.

Treatment⁵

• Avoid irritants and use a soap substitute.

• First apply a potent topical steroid (e.g. methylprednisolone aceponate)—continue until resolution of rash.

• Second (when controlled) apply LPC 2% in aqueous cream daily. If not tolerated, use ichthammol 1% in aqueous cream daily.

Note: Maintenance with topical steroids—hydrocortisone 1% or resume potent agent for a flare-up.

Lichen planus^6,7

Genital lichen planus is relatively uncommon but may affect both the vulva and vagina and can occur in association with oral lesions. It can also occur on any part of the skin, causing itchy skin rashes and nail dystrophy. Symptoms include irritation or vulval pain, dyspareunia and heavy but non-offensive vaginal discharge. Dyspareunia is usually severe.

Examination often reveals non-specific findings, ranging from subtle erythema to frank ulceration. Erosion of the mucosa surface of the introitus is a hallmark feature and may extend into the vagina and involve the cervix. If untreated, loss of the labia minor is typical and the clitoris may be buried by scar tissue.

Differential diagnoses of vulvar lichen planus include other causes of desquamative and erosive lesions, such as lichen sclerosus, pemphigus vulgaris, bullous and cicatricial pemphigoid and erythema multiforme.

A diagnostic biopsy is often difficult to obtain. Treatment is difficult and specialist input is required. Potent topical steroids provide symptomatic relief and there is a variety of treatment trials, including topical cyclosporin or oral methotrexate.⁵

Lichen sclerosus^4,5

Also known as lichen sclerosus et atrophicus (see FIG. 107.1), this uncommon chronic inflammatory dermatosis of unknown aetiology (perhaps an autoimmune disorder) presents as well-defined white, finely wrinkled plaques that almost exclusively affect the anogenital skin, although they can occur anywhere on the body. Lichen sclerosus spares the vagina. It can run a chronic and complicated course with development of squamous cell carcinoma (SCC) in about 4% a concern. The differential diagnosis is atrophic vaginitis.

Clinical features

• Bimodal peak: prepubertal girls, perimenopause

• Mean age of onset in adult women is 50 years

• Pruritus is main symptom

• Soreness, burning, dyspareunia

Examination

• Variable distribution

• White wrinkled plaques

• Purpuric and ulcerated areas (due to scratching)

• May show figure of 8 pale perianal and perivaginal area

Complications if untreated

• Vulval atrophy and labial (even clitoral hood) fusion, introital stenosis

• Lifetime risk of SCC 2–6%

Management⁵

• Best in consultation with a dermatologist.

• Confirm diagnosis by biopsy (tend to avoid in children).

• Based on potent topical corticosteroids (e.g. betamethasone dipropionate 0.05% in optimised vehicle ointment applied bd until itching ceases, then daily⁷—show patients where to apply, using a mirror).

• Goal of treatment is to return the skin to a normal colour and texture, which can take up to 6 months and unlikely if scarring has already occurred.

• A lower dose topical corticosteroid can be used for maintenance as soon as the white areas have resolved.

• Long-term treatment is required in 85% of postmenopausal women.

• Lifelong surveillance with 6-monthly check-up.

• A similar topical program is used in children.

• Surgery is reserved for complications or SCC.

Chronic vulvovaginal candidiasis

This is different from acute candidiasis and remains difficult to treat because there may be a localised hypersensitivity to Candida (see CHAPTER 106).

Clinical features

• Chronic vulval itch–scratch cycle

• Burning, swelling—premenstrual exacerbation

• Dyspareunia

• Discharge not usually present

• Aggravated by courses of systemic antibiotics

Management⁵

• Swab—low vaginal—with each suspected episode, especially if discharge

• Aim for symptom remission with continuous antifungal treatment:

  • – daily oral antifungals (monitor liver function tests) until symptoms clear—fluconazole 50 mg/day, or itraconazole 100 mg day (symptoms may remit in 2 weeks but often requires 6 months)

• Relieve itching with hydrocortisone 1% (do not use stronger preparations)

• Use nystatin in pregnancy

Tinea

Tinea causes an annular spreading rash with an active border that spreads from the labia to the thigh (see tinea cruris, CHAPTER 120). A problem is the development of tinea incognito from the application of topical steroids. This lacks central clearing but the active margin can be seen. Skin scrapings are necessary for diagnosis. Treatment is with a topical imidazole (avoid nystatin) or oral agents if resistant or extensive.

Pruritus vulvae

The causes of an itchy vulva to consider are:⁸

• candidiasis (rash, cottage cheese discharge)

• irritant dermatitis

  • – especially if excessive sweating and tight clothing

  • – sensitivity to soaps, bubble baths, cosmetics and contraceptive agents

  • – overzealous washing or wiping with toilet paper

• local skin conditions:

  • – psoriasis

  • – dermatitis/eczema

• post-anal conditions (e.g. haemorrhoids)

• infestations:

  • – threadworms (children)

  • – scabies

  • – pediculosis pubis

• infections (other than candidiasis):

  • – Trichomonas

  • – genital herpes, genital warts

• menopause: due to oestrogen deficiency (atrophic vaginitis)

• topical antihistamines

• vulval carcinoma

• psychological disorder (e.g. psychosexual problem, STI phobia)

If the labia minora are involved, consider lichen sclerosus. Treatment is according to the causation.

Management

This depends on the primary cause (e.g. candidiasis, incontinence), which should be treated effectively.

General measures (advice to patients)³

• Avoid overzealous washing.

• Take showers of no more than 5 minutes duration.

• Avoid having water too hot (lukewarm preferable).

• Use a soap substitute (e.g. aqueous cream, Cetaphil lotion) and wash it off with water only.

• Use soap alternatives (e.g. Dove, Neutrogena) for rest of body.

• Pat the skin dry after showering (avoid harsh drying).

• Do not wear tight pantyhose, tight jeans or tight underwear, or use tampons.

• Do not use vaginal douches, powders or deodorants.

• After the toilet, wipe gently with a soft, non-coloured, non-perfumed toilet paper.

• Apply a good moisturiser (e.g. Hydraderm or 5% peanut oil in aqueous cream).

Treatment

• For pruritus, apply cool moisturising cream (kept in refrigerator) when there is an urge to scratch.

• A zinc-based barrier cream or petroleum jelly may be appropriate if there is moisture irritation.

• Apply prescribed steroid ointment to the rash.

Vulvovaginitis is the most common gynaecological disorder of childhood. It can affect women of any age but is particularly common in girls, especially between the ages of 2 and 8 years. It is a type of dermatitis of the vulva and the vagina.

Mild vulvovaginitis

Symptoms of this very common problem include:

• discomfort and soreness

• vulval itching

• redness

• discharge—usually a slight yellow discharge on the underwear

• dysuria

It is important not to confuse it with a urinary infection when the child is clearly uncomfortable due to stinging on urination.

The cause is usually due to a low-grade inflammation in an area with a possible underlying dermatological disorder such as atopic dermatitis, psoriasis or lichen sclerosus, leading to sensitivity to various irritants such as soaps and urine.

Affected girls are often ‘atopic’.

The causal factors include:

• thin vaginal mucosa (the normal prepubescent state)

• dampness from synthetic-fibre underwear, tight clothing, wet bathers, obesity

• over- or under-wiping after passing urine

• frequent self-handling, especially with irritation

• irritants (soap residue, bubble baths, antiseptics, chlorinated water)

• ‘sandbox’ vaginitis: girls sitting and playing in sand or dirt may develop irritation from particulate matter trapped in the vagina

Management

• Explanation and reassurance to parents

• Avoidance of the above causal factors, especially wet bathers, synthetic underwear, bubble baths, perfumed soaps and getting overweight

• Attention to good, supervised toileting practice

• Attention to bathing and drying

It is worth soaking the child in a warm shallow bath containing half a cup of white vinegar.

Alternatively, bicarbonate of soda (10 g/10 L water) can be used.

Soothing creams such as soft paraffin creams and nappy rash creams such as zinc and castor oil cream should be applied three times daily as a short-term measure. If a powder is required, use zinc oxide (e.g. Curash). Consider an oestrogen cream.

Moderate/persistent vulvovaginitis

The symptoms may be more intense with increased itching, burning and discharge.

Important causes to consider⁹

• ‘Sandbox’ vaginitis

• Skin disorders, especially atopic dermatitis and lichen sclerosus (look for skin problems elsewhere on body)

• Foreign body: consider if a bloody, malodorous vaginal discharge

• Candidiasis—uncommon but consider if antibiotic therapy or possibility of diabetes

• Sexual abuse (uncommon but must not be missed)

• Pinworm infestation (Enterobius) (see FIG. 15.5 in CHAPTER 15)

• Sexually transmissible organisms—usually postpubertal

Examination

A careful general examination should be performed only if considered appropriate. In infants, the best examination method is to place the child on her parent’s lap with the legs held well abducted. Lateral traction applied to the labia allows the hymen orifice to be examined. Look for vulval or vaginal infection.

An older child can be placed in one of two suitable positions:

1. supine, legs apart in a frog-leg position, with bottom of feet touching (generally preferred)

2. prone, knee/chest position. This allows a better view of the hymenal orifice but many children do not like this position

A rectal examination may be performed to try to feel for suspected foreign bodies in the vagina.

Taking a swab⁸

If the discharge is profuse and offensive, take an introital swab (do not take a vaginal swab). Infective vulvovaginitis in girls is often due to a group A beta-haemolytic Streptococcus.

Treat with an appropriate antibiotic.

Treatment of dermatitis

Most cases of vulval dermatitis will respond to short courses of 1% hydrocortisone ointment or cream, provided aggravating factors are removed.

Labial adhesions (labial agglutination)

Labial fusion is caused by adhesions considered to be acquired from vulvovaginitis after which sometimes the medial edges of the labia minora become adherent. The adhesions are certainly not present at birth. Labial fusion is regarded as a normal variant and usually resolves spontaneously in late childhood. Provided the child is able to void easily, no treatment other than reassurance is needed.

For significant adhesions, treat as follows:

• oestrogen cream once a day until resolution (usually 2–6 weeks). Once the fusion has separated, ongoing treatment with soap avoidance, topical lubricants (e.g. Vaseline) and 1% hydrocortisone

• the fusion may re-form and have to be re-treated as necessary

Occasionally, a minor surgical procedure (sometimes under general anaesthetic) to separate the labial adhesions may be required. However, such measures are not generally recommended.

Vulvodynia describes the symptom of pain (burning, rawness or stinging) and discomfort, where no obvious cause can be found. Itch is not a feature. Causes include provoked vestibulodynia (vulvar vestibular syndrome), dysaesthetic vulvodynia and various infections (e.g. herpes simplex virus). Virtually every condition of the vulva can be painful at times; even dermatitis can become painful if scratching or splitting leads to open areas and ulceration.

Provoked vestibulodynia^2,4

Also referred to as vulvar vestibular syndrome (VVS) or vestibulitis, it is a very important disorder for the GP to be aware of in the woman with a typical history of introital dyspareunia. It is a difficult problem to treat. The characteristic feature is severe pain with vestibular touch, including attempted vaginal entry (early dyspareunia). The vestibule is very sensitive, featuring an inappropriate response to light touch. In many instances the cause of the primary condition is not apparent and a history of possible sexual abuse or other psychological provoking factors should be diplomatically elicited. Some patients can develop the problem after years of pain-free sex.⁶ It is the most common cause of dyspareunia in the premenopausal female.

Secondary causes include inflammatory triggers such as irritant contact dermatitis and infection. This establishes a conditioned response.

Spontaneous resolution has been reported in up to 50% of cases. Prognosis appears to be reasonably good but depends to some extent on the premorbid traits of the patient.³

Clinical features

• Delayed diagnosis (average 2–3 years)⁴

• Sexually active women in 20s and 30s

• Family history

• Pain provoked by intercourse, tampon insertion, tight underwear

• Superficial ‘entry’ dyspareunia

• Sexual dysfunction

• Tender vestibule on pinpoint light pressure

• Erythema (usually minute red spots) around Bartholin’s duct openings (consider Candida)

Diagnosis

Marked tenderness to light touch of the inner vestibule with a cotton bud.

Management⁴

• Investigate and treat if suspected underlying cause

• Patient education, counselling and support

• Multidisciplinary approach often required

• Physiotherapy—rehabilitation of pelvic floor musculature by increasing awareness and increasing elasticity of the tissues of the vaginal opening

• Counselling often beneficial, especially given impact on sexual health and relationships

• Genital skin care

• Encourage use of oil-based lubricants for intercourse

• Application of lignocaine 2% gel or 5% ointment to the vestibule 10–20 minutes before intercourse

Options¹⁰

• Biofeedback technique

• Tricyclic antidepressants (start low, e.g. amitriptyline 10–20 mg nocte then up to 100 mg—best option)

• Gabapentinoids (e.g. pregabalin starting at 75 mg or, if not tolerated, gabapentin starting at 300 mg)

• Intralesional therapy (no more effective than other treatments):

  • – triamcinolone

  • – botulinum toxin

• Vestibulectomy (role uncertain and controversial)

Dysaesthetic vulvodynia^2,11

The typical patient with this neuropathic pain problem is a middle-aged to elderly woman who presents with a constant burning pain of the labia, which typically builds up during the course of the day. Examination is often unrewarding. The underlying cause may be pudendal neuralgia (may be secondary to pudendal nerve block), referred spinal pain or simply unknown.¹²

Herpes simplex infection needs to be excluded.

Treatment options include antidepressants (TCAs and SNRIs) and gabapentinoids.

Bartholin’s cyst

A Bartholin’s gland swelling follows obstruction of the duct and presents as a painless vulval swelling at the posterior end of the labia majora, close to the fourchette. A simple, non-infected cyst can be left alone and may resolve spontaneously. If it becomes infected, an abscess may result, causing a painful, tender, red vulval lump. It may resolve with antibiotics or discharge spontaneously. Otherwise, drain and perform a micro and culture. The usual organism is E. coli. If the cyst persists and becomes large, a surgical marsupialisation procedure, which allows permanent drainage, can be performed (see FIGS 107.3A, B).

International Society for the Study of Vulvovaginal Disease <www.issvd.org>

Vulval Pain Society <www.vulvalpainsociety.org>