A combined clinic conducted by gynaecologists, dermatologists and genitourinary physicians has considerable merit (McCullough et al 1987). Facilities should include adequate lighting, a tilting examination chair to obtain access to the posterior part of the pudendum, a colposcope, and a camera for colpophotography and clinical photography.

Examination should consist of inspection of the vulva including the vestibule, urethral meatus, perineum and perianal area. Patients with neoplastic disorders must also have the cervix and vagina examined, but many older patients and those with lichen sclerosus will not tolerate a speculum examination and, unless there is any specific symptom, it is unnecessary to include this as part of the examination.

Colposcopic assessment of the vulva is not essential if there is an obvious, readily diagnosed lesion and there are no suggestions of neoplastic change. However, colposcopy is valuable if the patient is symptomatic but no lesion can be seen, if there is difficulty in interpreting or defining the limits of the visible lesions, and to select an appropriate site for biopsy. The techniques are described elsewhere (MacLean and Reid 1995). The vascular patterns can be complex and may be associated with neoplasia. Sometimes, sites of previous biopsies will develop unusual vascular patterns associated with healing. Excessive applications of potent topical corticosteroids will exaggerate vascularity with prominent telangiectasia and thinned epidermis.

Once the vulva has been scanned with the colposcope, aqueous acetic acid solution should be applied. It is important to realize that the aceto-white changes will not be as dramatic as those seen on the cervix, and may not be apparent in areas of abnormal keratinization. Aceto-white epithelium may represent vulvar intraepithelial neoplasia (VIN) or viral changes with human papilloma virus (HPV) and Epstein-Barr virus, as well as tissue repair with ulcers and erosions, scratch damage or coital trauma. It is incorrect to interpret the papillae seen in the vestibule as ‘microwarts’; Jonsson et al (1997) showed that aceto-white changes in the vulva were a poor predictor of viral presence.

In the past, histological diagnosis of the vulval lesions depended on inpatient biopsy performed under general anaesthesia. Very appropriate biopsy material can be obtained using a 4 mm diameter Stiefel disposable sterile biopsy punch, and this can be performed as an outpatient procedure under local anaesthesia (McCullough et al 1987). A silver nitrate stick with a small plug of cotton wool or, alternatively, ferric subsulphate (Monsel’s solution) is applied for haemostasis.

Prevalence of Vulval Lesions

Box 40.2 shows the range of lesions seen in a combined vulva clinic (MacLean et al 1998). There may be some bias in these figures because the clinic received referrals from gynaecologists, and because the authors’ research interests included vulval precancer and pain.

Box 40.2

Prevalence of vulval lesions seen in 1000 women

Lichen sclerosus	243
Dermatitis/lichen simplex	56
Lichen planus	23
Localized provoked vulvodynia	98
Generalized vulvodynia	32
Psoriasis	25
Diabetic vulvitis	9
Vulval Crohn’s disease	5
Acute contact vulvitis	2
Cicatricial pemphigoid	2
Plasma cell vulvitis	2
Neoplastic
Cancer	23
VIN, usual type	82
Paget’s disease	4
Melanoma	2

Source: MacLean AB, Roberts DT, Reid WMN 1998 Review of 1000 women seen at two specially designated vulval clinics. Current Opinion in Obstetrics and Gynaecology 8: 159–162.

Infections included recurrent candidiasis, bacterial vaginosis, trichomoniasis, herpes simplex virus, condyomata acuminata, ulcers associated with human immunodeficiency virus, hidradenitis suppurativa, Enterobius vermicularis (threadworms) and vulva lymphoedema secondary to filariasis, and were not enumerated because they sometimes coexisted with other conditions.

Use of Topical Corticosteroids

As many gynaecologists are apprehensive about the use of topical corticosteroids in the vulval area, some basic information is desirable.

Topical steroids are effective in the management of inflammatory changes but are contraindicated in the presence of untreated infection. In some situations, corticosteroids can be combined with an antifungal or antibacterial agent, but care must be taken with the latter because an allergic contact dermatitis can develop.

Topical preparations are suspended in a vehicle, usually as a cream or ointment. Creams are miscible with skin secretions, easy to apply smoothly (and sparingly) but may contain additive to which the patient will become sensitized. Ointments are usually insoluble in water, greasy in texture and therefore more difficult to apply and more occlusive than creams. As they encourage hydration, they are better suited for dry lesions. Pastes are stiffer but useful for localized lesions. They are less occlusive than ointments and can be used to protect excoriated or ulcerated lesions.

Topical corticosteroids are graded according to their potency, which reflects the degree of absorption or penetration through a lesion to be effective. A very potent steroid is necessary if there is lichenification or hyperkeratosis, or if the inflammatory changes are predominantly within the dermis. These include clobetasol propionate 0.05% (Dermovate) and diflucortolone valerate 0.3% (Nerisone Forte). Potent preparations include betamethasone 0.1% (Betnovate) and triamcinolone acetonide 0.1% (Adcortyl), moderately potent preparations include clobetasone butyrate 0.05% (Eumovate) and mild preparations include hydrocortisone 1%.

Patients will have anxieties about applying potent topical corticosteroids, particularly because some of the patient information with the packaging advises against their use in the genital area. This can be reduced by giving the patient written instructions on their use, either as an information sheet or in a letter with specific instructions for the patient.

Long-term use of potent corticosteroids does have side-effects which include the development of telangiectasia, striae, thinning of the skin, increased hair growth and mild depigmentation. However, clinical experience finds that this is rare for vulval applications, and is more likely when large amounts are spread over the buttocks or inner thighs. There is a risk of worsening infection if steroids are applied where yeast or fungal organisms are present. Systemic absorption is rarely seen. Side-effects can be reduced by using less potent preparations where possible. However, it is better to use more potent steroids to gain relief and then reduce either the potency or the frequency of application to maintain control. Sudden cessation will often produce a rebound of symptoms. A 30 g tube of Dermovate, if used appropriately [i.e. one application using a fingertip length (approximately 0.5 g) as squeezed from the tube] should last between 3 and 6 months.

On nights when topical steroids are not being applied or during the day, emollients can be applied to soothe and smooth the surface and to act as a moisturizer. Some emollients must be used with care in certain patients, such as those who have a history of irritation with wool, as components [e.g. wool fat (lanolin) or preservatives] can cause sensitization. Examples of emollients include aqueous cream BP, E45, Sudocrem, Ultrabase and Unguentum Merck (creams); Diprobase (cream or ointment); and emulsifying ointment BP, zinc and castor oil ointment BP (ointments). Some of these are suitable as soap substitutes (emulsifying ointment, aqueous cream), or preparations such as Alphakeri, Balneum or Oilatum can be added to the bath or shower (but can make the bath or shower dangerously slippery for the unsuspecting).

Dermatoses

While dermatologists will have little difficulty in reaching a diagnosis with these lesions, most gynaecologists are likely to be less certain. One of the confusing issues is the interchangeable use of the terms ‘eczema’ and ‘dermatitis’; essentially they mean the same thing. Dermatitis is found in 64% of patients with chronic vulval symptoms (Fischer et al 1995). Many of these patients will have skin manifestations elsewhere and will be identified by history and examination. The term ‘atopic dermatitis’ is used when there is clinical evidence of atopy (e.g. asthma, hayfever or allergic rhinitis in the patient or immediate family). In some cases, biopsy and histology will be diagnostic. Many lesions will respond to topical corticosteroids, although the required potency will depend on the diagnosis.

Contact and irritant dermatitis

Contact dermatitis occurs as an allergic response to various allergens including topical antibiotics, anaesthetic and antihistamine creams, deodorants and perfumes (e.g. Balsam of Peru as used in various haemorrhoid creams), lanolin, azo-dyes in nylons, biological washing powders, spermicidals, latex of sheaths or diaphragms, etc. An increasing number of referrals to vulval clinics are being seen because of the current trend to remove vulval hair. Some women have applied depilatory creams or waxes with subsequent irritant reaction, while others have inflicted skin damage by shaving.

Clinically, there is a diffuse erythema and oedema with superimposed infection or lichenification. Patch testing may identify the allergen to allow removal or avoidance of the factor, and moisturizing cream or mild steroids should provide local control. Haverhoek et al (2008) reported that 81% of the patients that they saw with vulval pruritus had at least one contact allergen detected on patch testing.

Some topical preparations will cause irritation over time (i.e. in a chronic fashion). The vulval appearances will be less obvious, with minimal erythema but with pallor or pigmentation, and with dryness, thickening and cracking — lichenification, or lichen simplex chronicus. Lichen simplex chronicus (previously known as ‘neurodermatitis’) occurs in normal skin which becomes thick and fissured in response to the trauma of constant scratching. Lichenification is a similar change which is superimposed on another pathology such as dermatitis. These lesions are not usually symmetrical and occur in vulval areas accessible to scratching.

Treatment consists of the use of emollients or topical corticosteroids of low-to-moderate potency. Sometimes, sedation at night is useful to stop nocturnal scratching. Once control is gained, assessment for an underlying cause or lesion is often necessary.

Psoriasis

Psoriasis may occur exclusively on the vulva, but often there are lesions elsewhere and a family history. It affects approximately 2% of the population. Unlike psoriatic lesions elsewhere on elbows, knees and scalp, they are unlikely to show the hyperkeratotic silvery scales but are often salmon-pink in appearance with a sharp but irregular outline and with satellite lesions. Vulval psoriasis is treated with mild or moderately potent topical corticosteroids.

Lichenoid lesions and vulval intraepithelial neoplasia

Terms such as ‘ichthyosis’, ‘leucoplakia’, ‘kraurosis’, ‘lichen planus sclereux’ and ‘dystrophy’ have created confusion in describing vulval lesions (Ridley 1988). The current terminology has evolved from the ISSVD’s 1976 classification of hyperplastic dystrophy, lichen sclerosus (no longer with ‘et atrophicus’) and mixed dystrophy; the abandonment of the term ‘dystrophy’ (MacLean 1991); and the use of the term ‘non-neoplastic epithelial disorder of vulval skin and mucosa’ (Ridley et al 1989) which included lichen sclerosus, squamous cell hyperplasia (formerly hyperplastic dystrophy) and other dermatoses. This did not cover those lesions with neoplastic potential or association, and the latest terminology incorporates ‘differentiated vulvar intraepithelial neoplasia’ for those lichenoid lesions with basal atypia and a significant association with vulval cancer (Sideri et al 2005, Scurry and Wilkinson 2006). This new terminology has met opposition, firstly because it removes the VIN 1, 2 and 3 classification which sat comfortably with CIN 1, 2 and 3 of the cervix, and replaces it with a single-grade system VIN (which groups together VIN 2 and 3 plus differentiated VIN). VIN 1 is uncommon compared with CIN 1. There is no evidence that it is a cancer precursor, and it is best considered as a warty lesion with no need for treatment and follow-up as one would do for the former VIN 2 and 3 lesions. The VIN frequently associated with HPV is now known as ‘usual or classic type’ (sometimes divided into basaloid and Bowenoid or warty subtypes, but this is not always meaningful because both subtypes are linked with HPV 16, and both appearances can occur in the same vulva). On the other hand, atypia or abnormality confined to the basal layer of lichen sclerosus (previously designated ‘hyperplastic/hypertrophic or mixed dystrophy with atypia’ may appear consistent with VIN 1 but has a significant association with vulval carcinoma, and thus differentiated VIN is regarded as high grade (Jeffcoate and Woodcock 1961, Leibowitch et al 1990, Scurry et al 1997). There are opinions that differentiated VIN is only found in the immediate vicinity when vulval cancer develops within an area of lichen sclerosus, but also a growing awareness of the basal cell changes (e.g. overexpression of p53, Ki-67 and CD1a) (Mulveny and Allen 2008) to provide the diagnosis in biopsies taken when cancer is not coexistent. Such cases are believed to have a greater risk of progressing to invasive cancer than cases with lichen sclerosus alone. Eva et al (2008) reported that there is an almost four-fold higher risk of recurrence when vulval cancer occurs in association with differentiated VIN.

Lichen sclerosus

The importance of lichen sclerosus is that it is common, occurring in at least one in 800 girls (Powell 2006) and increasing to one in 30 elderly women (Leibovitz et al 2000). It accounts for up to one-quarter of women seen in vulval clinics (MacLean et al 1998), 1.7% of patients seen in general gynaecological practice (Goldstein et al 2005) and 0.3–1 per 1000 of all new patients seen in a general hospital (Wallace 1971). It occurs in men, where it is known as balanitis xerotica obliterans, but at approximately one-sixth to one-tenth of the incidence in women. It is an inflammatory dermatosis and is frequently symptomatic, causing pruritus, sleeplessness, dyspareunia and constipation. It may produce major architectural changes and alteration in vulval appearance and function, and has a small but important risk of cancer; 60–70% of vulval squamous cell cancers occur against a background of or in association with lichen sclerosus. Lichen sclerosus involves the pudendum, either partially or completely as a figure-of-eight lesion encircling the vestibule and involving the clitoris, labia minora, inner aspects of the labia majora and the skin surrounding the anus. It is usually bilateral and symmetrical. It does not involve the vestibule or extend into the vagina or anal canal.

The lesions consist of thin, pearly, ivory or porcelain white crinkly plaques. Sometimes, there is marked shrinkage and absorption of the labia minora, coaptation of the labia across the clitoris to form a phimosis, and narrowing of the introitus to obscure the urethra and make intercourse impossible. Scratching will produce lichenification or may produce epidermal erosion and ulceration. Areas of ecchymoses and subsequent pigmentation are common. Lichen sclerosus may also involve the trunk or limbs in 18% of patients (Meyrick-Thomas et al 1988).

The histological features of lichen sclerosus typically show epidermal atrophy, dermal oedema, hyalinization of the collagen and subdermal chronic inflammatory cell infiltrate. There is a correlation between clinical appearance and histology, with the clinically thin area showing marked epidermal thinning with loss of rete ridges and vacuolation of the basal cells, while thick white fissured areas will histologically show hyperkeratosis, parakeratosis (abnormal keratinization) acanthosis and elongation, widening and blunting of the rete ridges (lichen sclerosus with lichenification; Ridley 1988). The inflammatory infiltrate may extend into the superficial dermis. These histological features are often modified secondary to trauma, with the presence of red blood cells or haemosiderin. However, histological changes may be minimal, and the histology may appear normal in some clinically obvious cases.

Cause of lichen sclerosus

The aetiology of lichen sclerosus remains unknown. Its uneven distribution between countries raises the possibility of an infectious cause (e.g. Borrelia burgdorferi spread by tick bites), but while there has been demonstration of these spirochaetes (Aberer and Stanek 1987), subsequent studies have not supported this theory.

The possibility of a hormonal (androgen-associated) cause has been examined without conclusive results (Friedrich and Kalra 1984, Kohlberger et al 1998). There is recognition that the incidence is higher among postmenopausal women, but no evidence that it is due to oestrogen deficiency. Young girls with lichen sclerosus seem to gain symptom improvement at puberty, but one study has documented that 75% of these teenagers have persistent features of lichen sclerosus (Powell and Wojnarowska 2002).

The suggestion that lichen sclerosus might be related to an autoimmune process is supported by finding associations with other autoimmune manifestations, a family history of autoimmune-related disease in first-degree relatives or the presence of circulating autoantibodies in patients with lichen sclerosus (Goolamali et al 1974, Meyrick-Thomas et al 1988). Recently, circulating basement membrane zone antibodies have been found in the serum of patients with lichen sclerosus (Howard et al 2004), and a specific circulating autoantibody to extracellular matrix protein 1 (ECM1) has been described (Oyama et al 2003). The nature of ECM1 epitopes in the serum of patients with lichen sclerosus has been examined, and an enzyme-linked immunosorbent assay has been developed which is highly sensitive and specific for lichen sclerosus, and discriminates between lichen sclerosus and other disease/control sera (Oyama et al 2004). Higher anti-ECM1 titres correlated with more longstanding and refractory disease and cases complicated by carcinoma. The factors that initiated this antibody are unknown, or its appearance may predate the development of lesions or symptoms. Investigation of the inflammatory process has demonstrated oxidative damage to lipids, proteins and DNA in biopsies taken from untreated patients with lichen sclerosus (Sander et al 2004). Interferon-γ has been demonstrated within lesional epidermis, underlying dermis and the zone of inflammation, along with other cytokines including tumour necrosis factor-α, interleukin-1α, IL-2 receptor CD25, intercellular adhesion molecule _1 CD11a and ICAM-1 ligand ICAM-1 (Farrell et al 2006).

Some supportive evidence for a genetic cause has been described (Marren et al 1995), and a link with human leukocye antigen (HLA) DQ7 has been demonstrated (Tasker and Wojnarowska 2003). Gao et al (2005) reported an increased frequency of DRB1*12 (DR12) and the haplotype DRB1*12/DQB1*0308/04/08/010, and a lower frequency of DRB1*0301/04 (DR17) among 187 women with lichen sclerosus, suggesting that HLA DR and DQ antigens or their haplotypes are involved in susceptibility and protection from lichen sclerosus.

Treatment of lichen sclerosus

Most dermatologists and gynaecologists now use topical steroids, such as clobetasol, and bland emollients to treat lichen sclerosus (Tidy et al 1996). However, it is only relatively recently that the effectiveness of topical steroids has been confirmed (Dalziel et al 1989, 1991, Lorenz et al 1998, Sinha et al 1999). Most patients will respond to nightly applications within a few weeks, but treatment can be continued for 1 month followed by alternate nights for the second month and twice weekly in the third month. A fingertip amount (approximately 0.5 g) is applied each time, and a 30 g tube of clobetasol should last for 3 months (Neill et al 2002). If patients do not improve, coexisting fungal infection, sensitization to the cream or coexisting carcinoma must be considered.

Renaud-Vilmer et al (2004) used a similar protocol for 83 women with lichen sclerosus, and calculated that the estimated incidence of remission at 3 years was 72% in women under 50 years of age, 23% in women aged 50–70 years and 0% in women over 70 years of age. The incidence of relapse was estimated to be 50% at 16 months and 84% at 4 years, and age was not a factor in relapse rate. Thus, discontinuation of treatment is likely to lead to a return of symptoms. Cooper et al (2004) reported on 327 patients managed in Oxford; 96% had symptom improvement with treatment (66% complete responses and 30% partial responses). Among the 253 patients who had clinical appearances recorded, 23% showed a total response with return to normal colour and texture, 68% showed a partial response, 7% showed a minor response and 2% showed a poor response.

Patients with squamous hyperplasia are less likely to respond (Clark et al 1999), but longer use or very potent steroids might be more successful. There is no advantage in applying oestrogen cream or testosterone ointment to the vulva.

Physical destruction by cryotherapy or laser ablation of areas of lichen sclerosus no longer seems justified, and nor is vulvectomy. Very occasionally, it may be necessary to divide labial or preputial adhesions.

Relationship between lichen sclerosus and vulval squamous cell carcinoma

This relationship continues to be an area of debate, and the positive side of the argument has been presented elsewhere (MacLean 1993, 2000a). Several authors have reported a prevalence of carcinoma of 2.5–5% in women who present with lichen sclerosus (Friedrich 1985, Meyrick-Thomas et al 1988). It is unlikely that the development of malignancy led to the appearance of lichen sclerosus, although many of these women denied having had symptoms for any duration. Nevertheless, there are individual cases or series of cases that document carcinoma occurring some time after a clinical diagnosis of lichen sclerosus, in spite of the use of appropriate clobetasol therapy. Jones et al (2004) reported that women who develop cancer with lichen sclerosus are more likely to show clinical evidence of squamous hyperplasia, and are more likely to have been symptomatic for 5 years or more (Jones and Joura 1999). Cooper et al (2004) reported that six women developed carcinoma in spite of treatment in their series. Renaud-Vilmer et al (2004) reported that among eight women who developed cancer, one had discontinued treatment 3 years earlier, and a second patient had used treatment irregularly.

There are concerns that certain treatments may increase the risk of cancer. There have been publications advocating the application of tacrolimus or pimecrolimus as alternatives to steroids, but following reports of apparently rapid progression to cancer, the US Food and Drug Administration released a warning advising caution with their use (Edwards 2008).

Many vulval cancers have lichen sclerosus in the adjacent skin, and the changes have been studied for various molecular alterations including changes in tumour suppressor gene activity and increased expression of mutant p53 (Kagie et al 1997, Kohlberger et al 1995), and allelic imbalance with loss of heterozygosity or allelic gain (Pinto et al 2000). The authors’ group have identified a mutation in codon 136 of exon 5 for p53 found in the areas of invasion and also in the adjacent lichen sclerosus (Rolfe et al 2003). Several patients with lichen sclerosus plus hyperplasia have progressed to carcinoma, and it is suggested that immunohistochemical staining of increased expression of p53 and Ki-67 may be a useful predictor of progression before invasion occurs (Rolfe et al 2001).

Until we have a better way of anticipating which patients with lichen sclerosus are at greatest risk of developing cancer, it is recommended that any patient who has difficulty with symptom control should be referred to a specialist/specialist clinic. This includes women who require topical potent corticosteroid application three or more times a week, or who use more than 30 g clobetasol in 6 months for symptom control. Women with clinical evidence of localized skin thickening/hyperkeratosis require biopsies. If the skin contains differentiated VIN or other features of concern, a short intensive course of topical treatment should be followed by review biopsies. Persistent areas of differentiated VIN require excision. Particular care is needed in the follow-up of patients who have already been managed for cancer, because their chance of recurrence is higher than for other patients with lichen sclerosus (Jones et al 2008).

The following mnemonic may be helpful when assessing lichen sclerosus:

Labial changes (e.g. reduction or fusion)

Introital changes (e.g. narrowing)

Clitoral changes including phimosis

Hyperkeratosis

Elevated or eroded areas

Neoplastic or suspicious area with hyperkeratosis or contour changes

Symptom control

Colour, usually with pallor but also ecchymoses

Localization, usually central

Extension posteriorly, causing anal symptoms and constipation

Rest of body; involvement elsewhere

Other autoimmune conditions or associated symptoms

Sexual difficulties

Urinary difficulties

Steroid use and misuse

Lichen planus

The lesions of lichen planus may be seen on mucous membranes or on cutaneous surfaces such as the inner surfaces of the wrists and lower legs. These cutaneous lesions are usually red or purple flat-topped nodules, or papules with an overlying white lacy-patterned appearance.

Involvement of the vulva is usually with white patterned areas that are sometimes elevated and thickened (hypertrophic lichen planus), or may appear red and raw with features of erosion. Changes in the mouth are often seen. The vulval lesions may extend into the vagina where scarring, stenosis and adhesions make intercourse painful or impossible. In a small number of cases, ‘crossover’ from lichen sclerosus (involving the vulval skin) to lichen planus (involving the vestibule and vaginal mucosa) occurs.

Histology will show liquefactive degeneration of the basal epidermal layer, long and pointed rete ridges, with parakeratosis, acanthosis and a dense dermal infiltrate of lymphocytes close to the dermal epidermal margin. Immunohistochemistry shows differences in the expression of interleukin-4 and interferon-α between lichen planus and lichen sclerosus (Carli et al 1997).

When the condition is severe, treatment can be difficult, requiring systemic steroids, azathioprine or other immune-modifying agents. Lesser symptoms, particularly those externally on the vulva, can be managed with the application of topical corticosteroids; vaginal lesions can be managed with Colifoam (hydrocortisone) or Predfoam (prednisolone). Rarely, vulval cancer will arise in association with lichen planus (Dwyer et al 1995, Zaki et al 1996).

Paget’s disease of the vulva

This is an uncommon lesion with uncertain malignant potential. Its appearance is of an eczematoid lesion with a scaly surface but vague margins, or it may be sharply bordered with a red and velvety texture, with areas or islands of hyperkeratosis. Histology classically shows large round atypical cells with oval nuclei and pale cytoplasm, singly or within clusters among the basal cells of the epidermis. These cells stain positively with cytokeratin 7, PAS para-aminosalcylic and CEA carcinomaembryonic antigen, and are regarded by many as carcinoma in situ. Their origin is uncertain. Their similarity to Paget’s cells seen in the nipple, usually with underlying breast carcinoma, questions their association with cancers. Studies of large numbers of vulval Paget’s cases report that less than 10% of cases will have an underlying adenocarcinoma of the vulva arising from an adnexal structure, and another 20% or more will have a carcinoma arising from adjacent viscera including rectocolon, endocervix or endometrium, ovary, urinary tract or breast. Wilkinson and Brown (2002) have proposed a classification that separates the lesions into those where the Paget’s lesion is in situ without any associated cancer, those with an underlying adenocarcinoma of a skin appendage or a subcutaneous gland, and those that are secondary to an underlying anorectal adenocarcinoma, a urothelial cancer or an adenocarcinoma arising from elsewhere (this latter group may be suspected by finding cytokeratin 20 expression in the Paget’s lesion). A small number of cases appear to progress from in situ to invasive adenocarcinoma if left untreated or undertreated.

Treatment by surgery is complicated with difficulty with primary closure, and plastic and reconstructive surgical techniques will be required. Achieving clear excision margins is difficult, and even when frozen section or definitive histology suggest clearance, recurrence will occur in 50% of cases or more. Currently, there is some enthusiasm for using topical imiquimod, although series are small and follow-up is short (MacLean 2000b, MacLean et al 2004).

Vulval Pain/Vulvodynia

Vulval pain is a common complaint in specialist vulval clinics, with many woman complaining that other doctors have either dismissed their symptoms or been unable to make a diagnosis or treat the condition effectively. However, the lack of clarity in the diagnostic categories reflects the complexity of the problem and the relative paucity of pathophysiological research in this area of medicine. Women with vulval pain are referred to genitourinary medicine, gynaecology, dermatology, urology, psychosexual medicine, physiotherapy and many other services. There are relatively few dedicated vulval clinics in the UK, and the majority exist to manage dermatoses and premalignant conditions, such as lichen sclerosus and VIN, rather than vulval pain.

Classification

The classification of vulval pain syndromes has been as difficult and confusing as vulval disorders in general; however, the most recent ISSVD classification logically uses a descriptive, anatomical and historical method. Vulval pain is not a single entity, and a detailed history and careful examination must be made before diagnosis is attempted. Vulval pain has been described in the medical literature for over 100 years (Skene, 1880). Previous definitions of these symptoms have referred to vulvar vestibulitis syndrome or burning vulvar syndrome, and to dysaesthetic vulvodynia. However, the lack of clarity in the diagnostic categories reflects the complexity of the problem, and the relative paucity of pathophysiological research in this area of medicine. Pain is described as either primary or secondary, provoked or unprovoked, and located anatomically, i.e. vestibulodynia, clitorodynia or the more generalized vulvodynia (Moyal-Barracco and Lynch 2004).

Pain characteristics

The predominant characteristic of vulval pain is a burning sensation. This can be accompanied by a description of ‘rawness’ or ‘soreness’. Provoked pain with entry dyspareunia as the primary complaint is usually described as a burning pain, and may be accompanied by superficial splitting or fissuring at the fourchette. The burning sensation may last several hours or even days after attempted intercourse. Similar pain may be associated with tampon insertion or withdrawal.

Unprovoked vulval pain is described as a constant unremitting sensation of pain, characteristically a burning or raw sensation that is not exacerbated by contact.

In clinical practice, there is inevitably a crossover of provoked and unprovoked pain, with women either having an element of ‘background’ pain or developing a complex pain syndrome with both provoked and unprovoked elements. Using the anatomical site of pain as a descriptor allows careful clinical examination, and will improve understanding of other urogenital pain syndromes such as interstitial cystitis.

Management

In younger women, the predominant symptom of dyspareunia is more commonly a secondary provoked vestibulodynia. Attempted coitus is accompanied by severe pain at entry that has a burning character and splitting of the skin at the fourchette. The pain may last for several hours or even days after contact. The appearance of the external vulval skin is normal, but there is a band of erythema in the vestibule; pressure from a cotton bud over the vestibular gland openings causes severe pain (Q-tip tenderness). This pressure can be quantified using an algesiometer, but is most often judged clinically (Eva et al 1999). It is helpful to define any triggers for this syndrome, particularly candida or bacterial vaginosis infections, or urinary tract problems. Local anaesthetic gel can be used on a cotton ball to ‘desensitize’ as well as to allow coitus to continue. Some patients respond to topical steroids, possibly because their symptoms are due to an underlying dermatosis. Pain-modifying drugs such as pregabalin, gabapentin or amitriptyline may be beneficial, particularly in longstanding pain or when there is a mixed picture of provoked and unprovoked pain. Increasingly, biofeedback techniques are utilized, ranging from pelvic floor physiotherapy (Glazer et al 1995) to cognitive behavioural therapy. Rarely, surgical removal of the vestibular skin is the treatment of choice. Reports of this procedure in the 1990s suggested a high level of success, but long-term follow-up data are less convincing. It should, however, not be dismissed as an option.

Unprovoked vulvodynia is rarely a primary complaint and is more common in older women. The characteristic burning is unremitting and is not influenced or exacerbated by contact. It is equated to other pain syndromes such as trigeminal neuralgia, and these patients may themselves have more than one pain syndrome. Treatment is with one or a combination of the pain-modifying agents amitriptyline, gabapentin or pregabalin.

The treatment of vulval pain requires a sympathetic, professional approach. The consultation needs to be long enough for the complex history to be explored fully and for patients’ anxieties to be discussed. Providing a diagnosis, no matter how limited, is reassuring. Simple measures such as advising the use of soap substitutes, managing precipitating factors and prescribing local anaesthetic gel are helpful. It may be worth considering physical therapies as described, but some women find using a transcutaneous electrical nerve stimulation machine beneficial. The use of pain-modifying agents means that patients must have clear advice about side-effects. It can be very helpful to work with colleagues in pain clinics, particularly in cases where there is clear evidence of pudendal neuropathy or in those cases where a combination of techniques is deemed necessary.

For a proportion of women, there is no resolution of pain despite all attempts at treatment. These women and many others require psychological support, and those that benefit from treatment may benefit from psychosexual counselling. Very little is known about how vulval pain affects partners, but anecdotally, couples report gaining benefit from counselling service. Women should be told about the patient support groups available in order to have the opportunity to talk to others similarly afflicted.

Vulval Ulceration

Vulval ulceration may be infective, aphthous or associated with Behçet’s syndrome, Stevens-Johnson syndrome, dermatitis artefacta, benign mucous membrane pemphigoid, familial benign chronic pemphigus (Hailey–Hailey disease), pyoderma gangrenosum, Crohn’s disease, histiocytosis X or toxic epidermal necrolysis (Lyell’s syndrome). These conditions are uncommon, but may be serious or even fatal. Sometimes, the diagnosis can be made clinically or with a good history. However, occasionally, biopsy will be necessary and may require immunofluorescent techniques.

Vulval Infection

Some lesions will be due to primary infection, while in other cases, a lesion has become secondarily infected. The commensal flora of the vulva consists of staphylococci, aerobic and anaerobic streptococci, Gram-negative bacilli and yeasts. Increased temperature, humidity and lower pH of vulval skin make it more susceptible to infection compared with skin elsewhere.

Fungal infections

Genital candida infection is caused by the yeast Candida albicans in the majority of cases. Occasionally, non-albicans species or yeasts such as Saccharomyces cerevisiae are identified, and these should be considered if there is limited response to the usual antifungal therapies. Candida are frequently found within the vagina, but incidence is increased with pregnancy, the use of oral contraceptives, the concurrent use of broad-spectrum antibiotics, the presence of glucosuria or diabetes mellitus, and in association with the wearing of nylon underwear and tights. Fungal infections are less likely to be seen in postmenopausal women unless they are diabetic or immunocompromised, and other causes of pruritus should be sought. Infection produces acute vulval pruritus associated with a crusting discharge, and white plaques will be seen within the vagina and on the vulva. With more extensive infection, the vulva will become acutely erythematous with oedema and superficial maceration. Culture on appropriate medium or direct microscopy will demonstrate the presence of fungus or hyphae.

Treatment of simple infection may be with a topical imidazole preparation (topical nystatin seems to have been discontinued). More extensive or recurrent infections can be treated with fluconazole 150 mg capsule as a single dose and repeated 1–2 weeks later, or itraconazole 200 mg morning and evening for 1–3 days.

Tinea cruris is relatively uncommon in females but may be transmitted from a partner; appropriate enquiry may be rewarding.

Genital warts

These are known as condylomata acuminata and are caused by HPV types 6 and 11 in the majority of cases. Descriptions of the role of oncogenic or high-risk HPV can be found in Chapter 38. Such lesions may involve not only vulval skin but also the vagina and the cervix, and may extend around the perianal area or out on to non-genital skin. Typical lesions are elevated with epithelial proliferation, usually discrete but sometimes confluent and covering large areas. The typical lesion shows koilocytosis in the upper third of the epithelium, acanthosis, parakeratosis, dyskeratosis and basal hyperplasia. The majority of condylomata acuminata are diploid, and only 10% may show nuclear atypia of various degrees, requiring differentiation from VIN with associated viral changes. The transmission of this virus is usually by sexual contact. The diagnosis is usually made on clinical appearance.

The treatment of single or small numbers of condylomata consists of the application of 25% trichloracetic acid followed by 25% podophyllin at weekly intervals. This combination should be applied to the lesion and the patient asked to bathe some 6–8 h later to remove any excess. Prolonged application can lead to excessive skin excoriation. Podophyllin should not be used during pregnancy. Those condylomata that are resistant to such treatment can be treated with imiquimod or some form of physical therapy. Atypical or resistant condylomata should be biopsied in order to exclude verrucous carcinoma.

Due to the widespread distribution of HPV and the difficulty in eradicating this virus from genital skin of immunosuppressed women, it may not always be appropriate to apply repeated, painful treatments that damage the vulval skin. For some, the repeated use of imiquimod may be useful. It is hoped that, in the future, the use of the quadrivalent vaccine, which will provide immunity against HPV 6 and 11, will reduce the clinical load from condylomata. Unfortunately, the recently introduced bivalent vaccine for British teenage girls is unlikely to prevent HPV 6 and 11 infection.

Herpes simplex

Vulval herpes lesions are usually associated with type 2 herpes simplex virus rather than type 1. The primary episode is associated with an incubation period of some 2–20 days, with an average of 1 week. There is often an associated prodromal illness before a localized area of vulval skin becomes erythematous, followed by the appearance of blisters and subsequent ulceration. These lesions are usually acutely painful and may be associated with inguinal lymphadenitis. Vulval discomfort associated with micturition may cause urinary retention. Secondary bacterial infection can occur. Resolution of discomfort and healing occurs some 7–14 days after the onset of symptoms. Frequently, the primary infection is much less dramatic. Following the primary episode, the virus enters the dorsal ganglia where it becomes dormant or latent. Reactivation may occur and a secondary episode results. Recurrences are less painful than primary lesions and rarely last more than 48 h.

The diagnosis and management of vulval herpes can be found in Chapter 63.

Bacterial infections

Bartholinitis

This is the most common bacterial infection of the vulva. It is usually found between menarche and menopause, but is not necessarily associated with sexual activity. It results from infection of the duct leading from the gland. Obstruction of the duct will produce an abscess in the acute situation, or a cyst if infection is low grade or recurrent. Causative organisms may be from the vulval flora or may be sexually transmitted.

The management of bartholinitis requires appropriate swabs for bacteriology, analgesia, antibiotics and surgical drainage of a cyst or abscess by marsupialization. In women over 40 years of age, the edges of the cyst should be sent for histological examination to exclude carcinoma.

Staphylococcal infection

This may take the form of perifolliculitis, involving the hair follicles and adjacent glandular structures within the dermis, or furunculosis which are larger, deeper lesions involving subcutaneous tissue that eventually discharge through multiple sinuses. Occasionally, staphylococcal impetigo is seen. Management consists of the application of povidone iodine washes or mupirocin ointment, or use of an antistaphylococcal antibiotic such as flucloxacillin. Occasionally, surgical drainage of the pus is required.

Hidradenitis

Similar features of recurrent staphylococcal infection are seen with hidradenitis suppurativa, a chronic inflammatory disease involving the apocrine glands (Thomas et al 1985). This condition is more likely to involve the axilla but will occasionally involve the vulva, perianal areas or the genitofemoral fold. These abscesses are deep and may often involve anaerobic organisms. Acute cases will require intravenous antibiotics such as flucloxacillin and metronidazole, and may require surgical deroofing of the abscess. Long-term antibiotic treatment may be combined with hormone control of apocrine gland activity. This appears to be best achieved using a combination of oestrogen and cyproterone. There is a small risk of carcinoma developing in areas of chronic scarring.

Other bacterial infections

Streptococcal infection of the vulva may be relatively superficial or localized, as in erysipelas. This will be associated with sharply defined erythema, oedema and pain. Streptococci may cause deeper infection involving tissue down to the fascia or periostium. Gangrene of the skin (necrotizing fasciitis) may complicate any form of vulval surgery, especially in diabetic women. High doses of penicillin are required. In cases with deep infection, other aerobic and anaerobic organisms are often involved, and a combination of antibiotics and very wide surgical excision is required. Infection of the vulva with Neisseria gonorrhoeae is rare in adults. The urethra, however, will be infected in approximately 75% of cases of gonorrhoea, and this may lead to infection of periurethral glands. Involvement of the duct of Bartholin’s gland is described above. Gonococcal vulvitis can occur in children.

Other bacterial infections involving the vulva include erythrasma, trichomycosis, tuberculosis (lupus vulgaris), syphilis, chancroid, granuloma inguinale and lymphogranuloma venereum, and is described in Chapter 63.

Protozoal and parasitic infections

Trichomonas vaginalis

This involves the vagina initially, although the patient may present with vulval symptoms. This infection is due to Trichomonas vaginalis, a flagellated protozoan, and is associated with vaginal discharge (which is often frothy and brown or green), vaginal irritation and dyspareunia. The organisms may be identified in a wet film by their motility or may be recognized in cervical smears. Colposcopic assessment of the vagina shows characteristic double-looped coarse punctation of the vagina and cervix, which may be diffuse and extensive or patchy and localized (strawberry vagina). Treatment of this infection is with metronidazole 200 mg three times a day for 7 days or a single 2 g dose. The partner should be treated concurrently and both should be warned not to take alcohol with this antibacterial agent.

Threadworms

Enterobius vermicularis live in the large bowel, lay eggs on the anal margins and cause pruritus ani; vulval irritation may also occur. The ova can be recognized on microscopy of a strip of Sellotape left in position overnight. Treatment is with piperazine or mebendazole.

Other infections that may involve the vulva include pediculosis (crabs), scabies, amoebiasis, schistosomiasis, filariasis and leishmaniasis. Vulval lesions due to entamoeba, schistosoma, filarial worms and leishmania are uncommon in Western countries. They should be considered in anyone returning from overseas, and may cause diagnostic difficulties if considering a ‘tropical’ venereal infection or even syphilis.

Benign Tumours of the Vulva

Lipomas and fibromas are the most common benign tumours of the vulva which arise from other than the epithelial tissues. Haemangiomas are benign tumours composed of blood vessels, with the capillary type (strawberry haemangioma) being most commonly encountered in infants and young children. A variety of site-specific stromal tumours can occur in the vulvovaginal area, including aggressive angiomyxoma, angiomyofibroblastoma and cellular angiofibroma (McCluggage 2009). In young to middle-aged women, fibroepithelial stromal polyps occur more frequently in the vagina, but also in the vulva or cervix. They are benign and are lined by normal squamous epithelium, which may be keratinized depending on the location; the stroma varies from bland to cellular and pleomorphic, especially in pregnancy-associated cases. There is potential for local recurrence, particularly if incompletely excised.

Angiomyofibroblastoma is a well-circumscribed lesion which occurs almost exclusively in the vulvovaginal region, and is often clinically thought to be a cyst of Bartholin’s gland. The stromal cells may have an epithelioid appearance and tend to cluster around vessels; they show reactivity for vimentin and desmin, and are actin negative.

Cellular angiofibroma occurs exclusively in the vulva of middle-aged women, and behaves in a benign fashion. It is composed of spindle cells arranged in short fascicles in a meshwork of thick-walled vessels. The cells express vimentin but not desmin or actin.

Aggressive angiomyxoma is a locally infiltrative tumour that recurs in 30–40% of cases if incompletely excised. It occurs most commonly in the reproductive years and usually has a gelatinous appearance. Histologically, it is a myxoid, poorly cellular neoplasm composed of bland spindle cells which merge imperceptibly into the surrounding stroma. There is no specific immunohistochemical marker, although high mobility group AT-hook2 HMGA2 expression may be useful.

Superficial angiomyxoma arises in the dermis and subcutaneous tissue, and therefore often appears polypoid. If multiple lesions involving other sites are present, Carney’s complex may be suspected and further investigations are warranted to exclude a cardiac myxoma.

Prepubertal vulval fibromas have been described which may be a childhood variant of aggressive angiomyxomas or may be enlargement secondary to hormonal changes approaching puberty. Superficial myofibroblastoma of the female lower genital tract may involve the vulva, vagina or cervix, are usually polypoidal or nodular, and are benign in behaviour (McCluggage 2009). Other smooth muscle tumours of the vulva are much rarer than the uterine counterpart; the presence of mitotic activity, nuclear pleomorphism or an infiltrative margin is associated with locally recurrent potential (atypical smooth muscle tumours), while tumours with any three of the following features are considered sarcomas: more than 50 mm in size, infiltrative margins, more than five mitoses/10 High-Power Fields, and moderate to severe atypia.

Granular cell tumours are of peripheral nerve sheath origin and arise in the vulva of children or adults as painless subcutaneous nodules of the mons pubis, labia majora or clitoris. They are often associated with pseudoepitheliomatous hyperplasia of the overlying epithelium, and are composed of epithelioid, granular cells infiltrating the stroma and typically exhibiting S-100 protein. Wide local excision is the treatment of choice. Neurofibromas may affect the vulva as part of von Recklinghausen disease. Benign angiokeratoma may be difficult to distinguish from a melanoma, especially if the initial red colour has given way to the later brown or black hue, and the lesion has started to bleed due to trauma.

Squamous papillomata and ‘skin tags’ are common, benign and similar in appearance. They may be solitary or multiple (vestibular or squamous papillomatosis, microwarts). They lack significant correlation with HPV DNA and histology may overestimate viral changes.

Seborrhoeic keratosis may also occur on the hair-bearing skin of the vulva of elderly women. The rare keratoacanthoma might well be mistaken for invasive squamous cancer because of its rapid growth over a matter of weeks. Spontaneous involution usually begins after approximately 6 months. The centre of this well-demarcated, regular dome contains a plug of keratin, which may suggest the diagnosis, but complete excision of the lesion is required for histological confirmation.

Vaginal Infection

Between puberty and the menopause, the presence of lactobacilli maintains a vaginal pH of between 3.8 and 4.2; this protects against infection. Before puberty and after the menopause, the higher pH and urinary and faecal contamination increase the risk of infection. Normal physiological vaginal discharge consists of transudate from the vaginal wall, squames containing glycogen, polymorphs, lactobacilli, cervical mucus, residual menstrual fluid and a contribution from the greater and lesser vestibular glands.

Vaginal discharge varies with hormonal levels and does not automatically mean infection. Non-specific vaginitis may be associated with sexual trauma, allergies to deodorants or contraceptives, or the chemical irritation of topical antimicrobial therapy. Infection may be aggravated by the presence of foreign bodies, continuing use of tampons and the presence of an intrauterine contraceptive device.

Vaginal infection may produce vulval symptoms, and the above descriptions of genital candidiasis, trichomoniasis, herpes simplex virus, HPV and syphilis are relevant to vaginal lesions. Neisseria gonorrhoeae will not infect the vaginal epithelium, except in prepubertal girls or postmenopausal women. If there is suspicion of sexual abuse in a young girl, an appropriate vaginal swab should be taken.

Bacterial vaginosis

Bacterial vaginosis is now believed to be due to a vibrio or comma-shaped organism named Mobiluncus. Other organisms, including anaerobes, may have a contributory role. These organisms are believed to be sexually transmitted, although the condition may be due to imbalance in the vaginal ecosystem. Usually, the vagina is not inflamed and therefore the term ‘vaginosis’ is used rather than ‘vaginitis’. Nearly half of ‘infected’ patients will not have symptoms (Thomason et al 1990), while others will complain of increased or unpleasant discharge, soreness and irritation.

Examination will reveal a thin, grey-white discharge and a vaginal pH greater than 5. A Gram stain of the discharge will show ‘clue cells’ which consist of vaginal epithelial cells covered with micro-organisms. The absence of lactobacilli will be confirmed if a characteristic fishy amine smell is released when a drop of vaginal discharge is added to saline on a glass slide, along with one drop of 10% potassium hydroxide. The diagnosis is made if three of these four criteria are met.

There are claims that bacterial vaginosis is associated with increased risk of preterm labour (Hay et al 1994), pelvic inflammatory disease and postoperative pelvic infection (Paavonen et al 1987, Eschenbach et al 1988). The treatment of bacterial vaginosis is metronidazole, either as 400 mg two or three times a day for 7 days or as a single 2 g dose. Alternatively, clindamycin 2% can be used as a vaginal cream but, unlike metronidazole, this is active against lactobacilli and will delay the restoration of normal vaginal flora.

Toxic shock syndrome

This topic has been included here because it is associated with the use of vaginal tampons during menstruation or less frequently in the puerperium (Shands et al 1980). Although there is a link between this syndrome and certain organisms (e.g. group A streptococci and Staphylococcus aureus) found within the vagina of affected women, it is not a vaginal infection. The manifestations are usually systemic with occasionally life-threatening consequences. Removal of superabsorbent brands of tampons from the market in the USA, and greater care in tampon use and insertion reduced the frequency of the syndrome. Early effective treatment of hypovolaemia in severe cases is essential. Treatment is the same as for septicaemia, and includes intravenous fluids and inotropic support where necessary. The cause should be eliminated where possible, and a β-lactamase-resistant penicillin should be given parenterally. Further attacks can occur and it is recommended that tampons should not be used until Staphylococcus aureus has been eradicated from the vagina.

Other Vaginal Pathology

Atrophy

This is seen following the menopause, but can also occur prior to puberty and during prolonged lactation. Examination shows loss of rugal folds and prominent subepithelial vessels, sometimes with adjacent ecchymoses. The patient may present with vaginal bleeding, vaginal discharge, or vaginal dryness and dyspareunia. Superficial infection, with Gram-positive cocci or Gram-negative bacilli, may be associated.

Treatment requires oestrogen to restore the vaginal epithelium and pH. This is usually by topical oestrogen cream or pessary. Systemic absorption will increase blood levels of oestrogen. Alternatively, hormone replacement therapy can be used.

Trauma

A torn hymen following a first attempt at intercourse may result in profuse, frightening haemorrhage. Suture of the bleeding vessel under general anaesthesia should be accompanied by one or more radial incisions in the hymen to prevent recurrence.

Trauma in young girls or women may be the result of falling astride an object like a bicycle or fence. It may result from sexual abuse, sometimes self-inflicted, but may represent sexual assault or rape. It may also occur following normal sexual intercourse, particularly in a postmenopausal woman who has not had intercourse for some time. In these cases, the laceration is usually at the vault of the vagina in the posterior fornix.

An indwelling catheter may be necessary and ice packs will give some comfort. Damage to the vagina, rectum, urethra, bladder or ureter may also occur. Pain relief with opiates and replacement of blood loss may be needed urgently. Examination under anaesthesia is often required to determine the extent of the damage. A closed haematoma is best managed conservatively, but bleeding lacerations will require suture. Devitalized tissues will need to be excised, and tears in bowel or bladder must be repaired in layers.

Seminal fluid allergy

Patients may present with a history of dyspareunia with itching, burning or swelling, or occasionally more widespread symptoms such as urticaria, bronchospasm or even angioneurotic oedema, after intercourse. The allergic reaction appears to be due to sensitization to seminal fluid (Poskitt et al 1995). Symptoms may arise following first coitus or after exposure to several sexual partners.

Treatment has included long-term immunotherapy following skin testing (Friedman et al 1984). Treatment with desensitization may be helpful, and antihistamines and intravaginal cromoglycate are described (Poskitt et al 1995).

Sjogren’s syndrome

Patients with vaginal dryness and pain may have Sjogren’s syndrome if there is ocular or oral dryness. They may have arthralgia, myalgia or Raynaud’s phenomenon. The diagnostic criteria include ocular and oral symptoms, ocular signs, salivary gland histology, other evidence of salivary gland involvement and the presence of autoantibodies. Vaginal symptoms may predate the oral or ocular symptoms.

Benign Tumours of the Vagina

Tumours in the vagina are uncommon. Condylomata acuminata are by far the most common type seen. The frond-like surface is usually characteristic, but it is wise to await the result of a biopsy before instituting treatment.

Endometriotic deposits may be seen in the vagina. They are most common in an episiotomy wound and may lie deep to the epithelium.

Simple mesonephric (Gartner’s) or paramesonephric cysts may be seen, especially high up near the fornices. If asymptomatic, they are best left untreated. If treatment is required, marsupialization is effective and safer than excision. Similarly, management of anterior wall cysts must include the exclusion of a bladder or urethral diverticulum.

Adenosis (multiple mucus-containing vaginal cysts) is a rare condition which, even more rarely, gives rise to symptoms. A variety of abnormalities are reported in the daughters of women who took diethylstilboestrol during their pregnancy. Most of these are of no significance (see Chapters 38 and 39).

Female Circumcision/Genital Mutilation

It is inevitable that obstetricians and gynaecologists will encounter patients with female genital mutilation (FGM), and management will sometimes cause more harm than good. Gordon et al (2007) emphasized that reversal procedures are better performed in non-pregnant or antenatal patients, rather than when the patient presents in labour and is dealt with by junior or inexperienced staff.

The World Health Organization (http://www.who.int/topics/female_genital_mutilation/en/) has defined FGM as all procedures involving partial or total removal of the external female genitalia, or other injury to the female genital organs for non-medical reasons. FGM is classified into four types, as follows.

• Type I: partial or total removal of the clitoris and/or prepuce (clitoridectomy).

• Type II: partial or total removal of the clitoris and labia minora, with or without excision of the labia majora (excision).

• Type III: narrowing of the vaginal orifice with creation of a covering seal by cutting and appositioning the labia minora and/or the labia majora, with or without excision of the clitoris (infibulation).

• Type IV: all other harmful procedures to the female genitalia for non-medical purposes; for example, pricking, piercing, incising, scraping and cauterization.

Most of the cases described by Gordon et al (2007) and managed in North London were of type III and the clitoris was found intact after reversal.

It is estimated that more than 2 million women and children undergo some form of FGM each year, mainly in Africa, but also in the Middle East and Asia. It is a cultural rather than a religious requirement or duty. Performing FGM in the UK became illegal in 1985, and in 2003, it became an offence for UK citizens to perform FGM in other countries. Nevertheless, many young girls appear to leave the UK to have the procedure performed between their birth and reaching puberty. Acute complications include pain, infection, bleeding, injury to the urethra/vagina/rectum, urinary retention, faecal incontinence and death. More chronic complications include urinary and menstrual difficulty, haematocolpos, sexual difficulty if not impossibility from fibrous scarring, and epidermoid cysts. Incompetent attempts at defibulation have caused urethral and rectal injuries, further scarring and persistent dyspareunia.

As mentioned above, Gordon et al (2007) advocated reversal as an elective procedure rather than during labour. If it is performed during labour, the fused labia minora should be incised in the midline anteriorly as far as the urethral meatus, and further anterior dissection of clitoral structures should be deferred because of risk of urethral damage and difficult haemostasis. It is illegal to close the infibulations following delivery, even if the husband insists. When performed electively, the CO₂ laser provides a bloodless incision, but care must be taken of underlying structures. Examples have been seen where careless use of electrodiathermy has left damage to underlying clitoral glans when dissecting a paraclitoral epidermoid cyst.

Gordon et al (2007) emphasized the requirement of providing an interpreter and nurse/midwife who will meet the psychosexual and social aspects of FGM. Certainly, the scenario of a patient presenting in advanced labour with undisclosed infibulated labia and the threat of imminent tearing should be avoided.

KEY POINTS

1 A multidisciplinary team approach to vulval disease is helpful.

2 The presenting symptoms will not indicate the nature of the pathology.

3 Topical steroids are effective and safe in the treatment of many vulval disorders.

4 Topical oestrogens have little to offer the woman with a vulval disorder.

5 There is no place for vulvectomy in the management of lichen sclerosus.

References

Aberer E, Stanek G. Histological evidence for spirochetal origin of morphoea and lichen sclerosis et atrophicus. American Journal of Dermatopathology. 1987;9:374-379.

Belfiore P, Di Fede O, Cabibi D, et al. Prevalence of vulval lichen planus in a cohort of women with oral lichen planus: an interdisciplinary study. British Journal of Dermatology. 2006;155:994-998.

Carli P, Moretti S, Spallanzani A, et al. Fibrogenic cytokines in vulvar lichen sclerosus. Journal of Reproductive Medicine. 1997;42:161-165.

Clark TJ, Etherington IJ, Luesley DM. Response of the vulvar lichen sclerosus and squamous cell hyperplasia to graduated topical steroids. Journal of Reproductive Medicine. 1999;44:958-962.

Cooper SM, Gao XH, Powell JJ, Wojnarowska F. Does treatment of vulvar lichen sclerosus influence its prognosis? Archives of Dermatology. 2004;140:702-706.