Vulval disease is a difficult area of women’s health, and as a result is often neglected or overlooked. Assessment of vulval disease is particularly difficult in general practice where specialist equipment or even decent lighting may not be available and all too frequently examination of this area is omitted entirely from the consultation. A combination of factors including patient embarrassment or the assumption by both the woman and the clinician that all vulval symptoms are attributable to thrush can often lead to serious and potentially avoidable pathologies being missed. Vulval disease affects women of all ages and the golden rule is that if a woman has persistent symptoms that are not responding to antifungal medication, then it probably is not candida and she needs a proper examination.

Vulval disease has a major impact on women’s lives and can have particular effect on sexual relationships. Early diagnosis and appropriate treatment or referral can alleviate suffering that otherwise may persist for many years.

The normal vulva

The vulva comprises the mons pubis, labia majora, labia minora, clitoris, vestibule of the vagina, bulb of the vestibule, and the greater vestibular glands. Diseases of the vulva may involve all or part of the vulva but it is important to remember that certain pathologies may manifest themselves elsewhere in the body as well.

The appearance of the normal vulva changes throughout life and is affected by different physiological factors. Children with vulval problems are often assumed to be the victims of abuse, and although these problems may coexist, false accusations can easily be made without considering possible pathology. Pregnancy and subsequent childbirth will alter the appearance of the vulva and vulval varicosities are more common during this time. Episiotomy or birth trauma may lead to changes in the vulval appearance that are not necessarily pathological, although referral may be appropriate. The practice of female genital mutilation (female circumcision) in some countries of Africa is common and, depending on the extent of the mutilation, persistent vulval problems may occur. Atrophy of the tissues at the menopause is often attributed to many vulval symptoms described by older women; diagnosis prior to examination should be resisted.

Clinical features of vulval disease

The two most common symptoms are itching or pain. Pruritus is probably the most common symptom and causes of vulval pruritus are found in Table Causes of vulval pruritus. Pain is often described as soreness or a burning sensation and may or may not be related to intercourse. Often the initial presentation is prompted by difficulties with sexual intercourse. Pain at penetration, splitting of the vulval skin or postcoital burning are often described, as are problems with insertion or removal of tampons. Specific questions should be asked about these problems, as many women, and particularly older women, are often embarrassed and will not volunteer such information, or the clinician assumes the woman is not sexually active.

The woman may have discovered a lump or ulcer, or noticed changes in the colour of the vulva as pigmentation or pallor are common findings. Other skin conditions are relevant as is a smear history, as we know that certain conditions are related. A proportion of vulval lesions are asymptomatic and found incidentally at examination.

Adequate examination of the vulva may be difficult in general practice, especially in immobile patients and some lesions may not be seen with the naked eye. Examination should include the whole of the perineum extending up to the crural folds and posteriorly to the perianal area. The groin should be examined and lymph nodes palpated. Other areas of skin and the buccal mucosa may be involved and should also be examined, if appropriate.

Some of the physical changes seen in vulval disease are fairly obvious, whereas other changes may be more subtle. Commonly seen signs are changes in the pigmentation of the skin, either pallor or deposition of pigment. Lichenification or thickening of the skin may be part of the pathological process or as a result of scratch damage secondary to symptoms. Changes may be symmetrical (as in lichen sclerosus) or unilateral with a specific discrete lesion. Erythema may be generalized (in vulval candidiasis) or local (in vulval vestibulitis syndrome). Changes in the vulval architecture may be apparent and splitting or fusion of tissue is often visible. Tenderness may be elicited over different areas of the vulva and this should be noted.

Women who present with suspicious looking areas should be referred urgently.

Table Causes of vulval pruritus

  Clinical features Treatment
Fungal overgrowth Red hot beefy vulva Topical antifungals В± oral fluconazole
Lichen simplex Thickened, flaky skin Topical steroids and emollients
Scratch damage or splitting  
Psoriasis Symmetrical red scaly Topical steroids, avoid tar products
Eczema Scaly thickened skin Topical steroids
Dermatitis (allergic or contact) Erythematous, oedematous skin Remove offending irritant
May blister May need patch testing
Topical steroids
Lichen planus Erosive or hypertrophic lesions Topical or systemic steroids
Desquamative vaginitis, ulceration or stenosis Retinoids
  Immunosuppression
Wickham’s striae in mouth  
Hygiene Excoriated, erythematous, oedematous tissue Improve hygiene
Emollients for symptomatic relief
Atrophy Loss of glycogenated tissue Hormone replacement therapy
Dry vagina  
Medical disorders Varied: e.g. in association with diabetes, chronic renal failure Treat underlying condition
Lichen sclerosus Atrophic or hypertrophied skin Topical steroids
Symmetrical pallor, loss of architecture. Ecchymosis Biopsy suspicious areas
Surgery only for adhesions or malignancy
Vulval intraepithelial neoplasia Warty lesions Excision and regular surveillance
Unifocal or multifocal  
May involve anus  
Vulval carcinoma Ulcerated lesion Surgery
Hypertrophic lesion  
May coexist with lichen sclerosus or VIN  
Paget’s disease Erythematous, scaly lesions Surgery

Psychological features of vulval disease

Vulval disease can have a massive impact on women’s lives, both physically and psychologically. Many vulval patients, particularly those with vulval pain syndromes, are labelled “mad” as there are no obvious physical signs, and although there is no doubt that psychological factors play a large role in the understanding and treatment of these conditions, many of these are a result of the physical condition and not the cause.

The vulva is a sexual organ and conditions that affect physical appearance and function will have detrimental effects on sexual relationships, leading to other relationship difficulties. Both disease and treatment, be it surgical or radiotherapy, can be disfiguring and/or impair sexual function. Studies have looked at psychosexual dysfunction in women undergoing vulvectomy for vulval cancer and found distress levels compatible with other cancer sufferers; dysfunction was both physical and psychological and manifested itself more severely in younger and single women.

Non-malignant conditions can be equally distressing and destructive. Adhesion formation and fusion of the labia with lichen sclerosus and obliteration of the vagina with lichen planus severely affect sexual function. Often this aspect is overlooked, as these conditions tend to affect the older population, who are often assumed not to be sexually active. It is therefore vital to explore this avenue in consultation.

Work looking at the psychological aspects of vulval pain has confirmed the need for a prompt diagnosis, as labelling the problem is often the first step to resolution. Women with vulval pain syndromes have significantly increased psychological morbidity compared with asymptomatic controls and within the category of vulval pain, women with dysaesthetic vulvodynia (where there are no physical findings) have the greatest levels of psychological stress, compared with women with other vulval diseases. These women are highly aware of their body image, are more likely to consult their doctor about other symptoms, and have increased levels of somatization.

Often these women have had symptoms for many years and are trapped in a vicious circle to the extent that even when the physical problem is resolved, they are unable to resume a normal life. Vaginismus is commonly secondary to the initial disorder and the role of psychosexual counsellors and physiotherapists with an interest in the pelvic floor is important. In women with persistent symptoms, coping mechanisms, pain management strategies, and support groups may help.

Because of the potential long-term problems that vulval disease may cause, referral to a multidisciplinary vulval clinic is ideal, although not always available, rather than to routine gynaecology or dermatology out-patients.

The vulva clinic

Vulval disease overlaps the areas of gynaecology, dermatology, and genitourinary medicine. Ideally, referral of patients from primary care should be to a specialist vulval clinic that is multidisciplinary.

Prior to referral it is important that the primary care doctor has examined the patient. Often vulval clinics have long waiting times for first appointments, but if a potential malignancy is suspected, then urgent referral is paramount. High vaginal and endocervical swabs may be performed prior to referral to eliminate any infective cause that can be treated easily. Blood tests generally are of no value.

Examination of the vulva should be conducted in a private area, preferably a separate room and not a curtained-off area. Adequate lighting should be available. Optimum examination is in the lithotomy position and a tilting chair is ideal to achieve this.

Colposcopy is increasingly being used in the assessment of vulval disease and closed circuit television to be attached so the patient can identify problem areas. KY jelly may be used to minimize the reflection of light in hair-bearing areas. As in cervical colposcopy, acetic acid may be used to identify lesions. Toludine blue may also be used to define areas of abnormality. Swabs may be taken to exclude infective causes of acute vulvovaginitis, e.g. Candida, gonorrhoea, Trichomonas, bacterial vaginosis.

Biopsy is easily performed in an out-patient setting. Local anaesthetic is injected using a dental syringe and punch biopsy may be obtained using a 4 mm Stiefel punch. Haemostasis is achieved using silver nitrate sticks or Monsel’s solution and sutures are rarely required.

Diseases of the Vulva

Vulval Disorders: Non-Neoplastic Disease

Neoplastic disease

Vulval intraepithelial neoplasia

Previously known as Bowen’s disease, carcinoma in situ, dystrophy with squamous atypia, vulval intraepithelial neoplasia (VIN) is a premalignant condition of unknown aetiology, although there are strong associations with smoking and human papillomavirus (HPV). vulval intraepithelial neoplasia affects a wide age range with the peak incidence in women in their forties. There is an association with cervical intraepithelial neoplasia (CIN) in 20 – 50% of cases.

Vulval intraepithelial neoplasia is recognized as a premalignant condition; however, there is debate as to the risk of progression. Originally thought to be around 5%, but greater in older women, it has now been suggested that the rate may be much higher. Equally, vulval intraepithelial neoplasia may regress in a similar fashion to cervical intraepithelial neoplasia. For these reasons patients need careful surveillance and regular follow-up, usually under hospital care.

Clinical features

vulval intraepithelial neoplasia has variable presentation and is often mistaken for vulval warts. The most common symptom is pruritus, but patients may present with bleeding, dyspareunia, or have noticed skin changes. Lesions may vary in colour, ranging from white to pigmented red-brown. There may be a discrete lesion or multifocal areas extending over the vulva and perianal area. Abnormal areas may be visualized more easily under colposcopic examination with the application of acetic acid or toludine blue. It is important to exclude multifocal intraepithelial neoplasia and therefore the cervix and vagina should be examined carefully. Persistent pruritus despite treatment should always be treated with suspicion.

Treatment

Regular surveillance of these patients is important, even after surgical treatment, as recurrence may occur. Symptoms may be controlled with potent topical steroids. vulval intraepithelial neoplasia 3 is treated by wide local excision, or, in the case of extensive disease, skinning vulvectomy using a laser may be appropriate.

Vulval cancer

Vulval carcinoma is a relatively uncommon cancer of the female genital tract, accounting for less than 5% of genital tract malignancies. It most commonly affects older women. Ninety per cent of tumours are squamous cell carcinomas, the remainder being malignant melanomas or basal cell carcinomas.

Squamous cell carcinoma

Vulval cancer may occur spontaneously, but is often associated with a pre-existing condition such as vulval intraepithelial neoplasia or lichen sclerosus. Spread is local and then to the lymphatics with late spread to the lungs and liver. Prognosis depends on the depth of invasion and nodal involvement. Overall 5-year survival is 75%. If there is no nodal involvement, this increases to 90 – 100%; if there is inguinal node involvement, survival is 30 – 70%; the involvement of pelvic lymph nodes significantly reduces 5-year survival to less than 25%.

The International Federation of Gynaecology and Obstetrics (FIGO).

Clinical features

Vulval cancers are often not discovered until they are advanced. This is either due to late presentation by the patient, often due to embarrassment, or by failure of the clinician to examine an older woman with persistent vulval pruritus and blind treatment, assuming the symptoms are due to candida infection. Therefore it is imperative to examine any woman with persistent symptoms. The most common symptom is pruritus, although vulval carcinoma may present with bleeding, ulceration, lump, pain, or abnormal discharge.

Treatment

Histological diagnosis should be obtained prior to treatment. It is now generally agreed that stage I and II cancers can be treated with wide local excision and do not need radical surgery. Lateralized tumours initially only need dissection of ipsilateral lymph nodes; the contralateral nodes are taken only if the initial nodes prove positive (Royal College of Obstetricians and Gynaecologists). Stages III and IV are treated with radical vulvectomy with lymph node dissection; this is normally now performed through separate incisions and the traditional “butterfly” incision is seldom used. If there is lymph node involvement, then external beam radiotherapy is used. Chemotherapy is not routinely used.

Other vulval malignancies

Malignant melanoma

Melanoma accounts for approximately 10% of vulval malignancies and has a poor prognosis, as it has often spread before presentation. Peak incidence is in the sixth and seventh decades of life.

It usually presents as a pigmented lump that is growing rapidly. There is a move to treating with local surgery, as more radical surgery does not appear to improve survival. Five-year survival is 8 – 50% and, as with other melanomas, a Breslow depth of more than 0.76 mm worsens prognosis. Metastases may occur years after treatment.

Basal cell carcinoma

Basal cell carcinoma accounts for 2 – 4% of vulval malignancies and is usually found on the anterior labia majora, although they may occur elsewhere. Metastasis is unusual and treatment is by local excision, although about 20% may recur.

Adenocarcinoma

Adenocarcinoma of the vulva is very rare.

Paget’s disease

Extramammary vulval Paget’s is usually found in postmenopausal women. Unlike breast disease, there is only underlying adenocarcinoma in about a quarter of cases. About 10% will have carcinoma elsewhere, e.g. breast.

Again the most common symptom is pruritus, which may take years to develop and so a large proportion will be asymptomatic. Lesions are usually erythematous and scaly with sharp demarcation and raised edges. Management involves wide excision, as progression to invasive carcinoma is rare, although recurrence may occur.

Vulval Pain

Vulval Disorders: 10-Minute Consultation

Vulval Disorders: Frequently Asked Questions

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