57 Fiona M. Lewis1,2 1 St John’s Institute of Dermatology, Guy’s and St Thomas’ NHS Trust, London, UK 2 Frimley Health NHS Trust, Slough, UK Many women who present with vulval symptoms may have a dermatological problem rather than an infective or gynaecological complaint. It is therefore important that all healthcare professionals who see these patients learn to recognize the common skin conditions that affect the vulva and to refer for dermatological advice and management appropriately. Many clinics with a multidisciplinary team have been established to provide a specific service for women with vulval disorders. This chapter gives an overview of the common skin problems that affect the vulva and the basic principles of initial management. A good history is the initial step in making an accurate diagnosis in any patient who presents with vulval symptoms. A clear method of history‐taking should be used, and a proforma can be helpful to ensure that the key areas are covered. The interview should take place in a sympathetic environment and it is often helpful to enquire about the general history initially before moving on to the more personal questions related to vulval disease. The following areas should all be covered, but sometimes more detail is required about specific symptoms depending on the presenting problem. The examination should always be carried out with a trained chaperone present. Good lighting and appropriate magnification is essential. The vulva can be adequately examined with the patient in the dorsal and left lateral position. Again, the examination should be approached systematically. The vulva is first examined overall but the labia majora need to be separated in order to adequately visualize the internal structures of the vulva. The vagina and cervix should be examined in all patients with erosive disease (e.g. erosive lichen planus, autoimmune bullous disease) and vulval intraepithelial neoplasia, as well as in patients complaining of a vaginal discharge. An examination of the skin at extragenital sites will often give valuable diagnostic information and inspection of the oral mucosa, eyes, scalp, nails and other flexural sites is important. There are some very common and important normal variants seen on examination of the vulva. These should be easy to recognize and the patient reassured. There are also physiological changes that vary with age and hormonal status. These are common and are usually seen on the labia majora. They are often multiple and appear as small red or purple vascular lesions with overlying hyperkeratosis (Fig. 57.1). Fig. 57.1 Angiokeratomas: dark‐red papules seen on the labia majora. (See also colour plate 57.1) This demarcates the junction of the keratinized and non‐keratinized epithelia of the labia minora and vestibule, respectively (Fig. 57.2). It can be very prominent in some women. Fig. 57.2 Hart’s line demarcating the junction between the keratinized skin of the labia minora and the non‐keratinized mucosa of the vestibule. (See also colour plate 57.2) A common finding is the presence of tiny filiform projections on the inner labia minora and vestibule (Fig. 57.3). Originally, it was thought that these were related to human papillomavirus (HPV) but there is good evidence that this is not the case. They are a normal variant and do not require any treatment. Fig. 57.3 Vestibular papillae: filamentous projections of the vestibular epithelium. (See also colour plate 57.3) These are small sebaceous papules found on the inner surfaces of the labia minora (Fig. 57.4). They can be very prominent in some women and may also be seen on the buccal mucosa. Fig. 57.4 Fordyce spots: tiny yellow papules on the inner labium minus. (See also colour plate 57.4) In the first few weeks of life, the vulva is under the influence of maternal hormones. The clitoral hood and labia minora are relatively prominent and may be seen without separation of the labia majora. Labial adhesions are common and usually resolve with time. Topical oestrogens may be helpful. Deposition of fat increases the size of the labia majora and mons pubis, and pubic hair appears. The rims of the labia minora may become pigmented. The clitoris enlarges and the vestibular glands become active. The vulva can be engorged and varicosities are common. Hyperpigmentation can be significant. The labia majora become less prominent and there is a reduction in hair growth. In some cases, a firm diagnosis can be made on the clinical appearances alone, but in others a variety of investigations are needed to confirm the diagnosis. This is a very simple procedure that can be performed in the outpatient clinic under local anaesthesia. A 4–6 mm punch biopsy is taken after infiltrating the area with lidocaine. Topical EMLA (lidocaine 2.5%, prilocaine 2.5%, Astra Zeneca, London, UK) may be used before injecting the lidocaine but care must then be taken in interpreting the histological appearances as subepidermal cleavage can be induced by this agent [1]. Clinicopathological correlation is vital in all cases of vulval dermatoses, and review by a dermatopathologist is very helpful. In cases where an autoimmune bullous disorder is suspected, a biopsy should also be taken for direct immunofluorescence. Appropriate swabs and transport media for bacterial, yeast and viral culture may be needed. If a sexually transmitted infection is suspected, the patient should be referred to a genitourinary clinic for full investigation and contact tracing. Skin scrapings to look for fungi can be taken in the clinic and direct examination of the skin under Wood’s lamp may be helpful to confirm erythrasma, where the affected skin will fluoresce pink. This is performed where an allergic contact dermatitis is suspected, either as a primary problem or where it is a secondary phenomenon caused by an allergy to treatment. Patients need to be referred to a dermatologist for these tests, which should include specific allergens that may cause problems on the vulva. Lichen sclerosus is an inflammatory dermatosis with a predilection for the anogenital skin [2]. It is significantly more common in women than men. Extragenital lesions may be seen in about 10% of women with genital involvement. They present as ivory white plaques, often at sites of trauma or friction (Fig. 57.5). Fig. 57.5 Extragenital lichen sclerosus: ‘white spot disease’. Flat white lesions which can coalesce into plaques. Follicular plugging may be seen. (See also colour plate 57.5) The aetiology remains unclear but it is thought that it is mediated by a lymphocyte reaction. Immunohistochemical alterations of the epidermis and dermis support an autoimmune cause and circulating IgG antibodies to extracellular matrix proteins have been demonstrated. There is an association in both the patient and their first‐degree relatives with other autoimmune diseases, particularly thyroid disorders. There are two peaks of presentation: in childhood and around or after the menopause. The predominant symptom is that of pruritus but soreness and dyspareunia will be experienced in the presence of ulceration, erosions and fissures. In children, constipation is a frequent feature if the perianal area is affected. The early lesions are white ivory papules that may coalesce to form plaques. Ecchymosis due to rupture of dermal vessels is common, as is oedema (Fig. 57.6). Ecchymosis is common in children and often leads to the erroneous diagnosis of sexual abuse. Extension of disease around the perianal area presents as a ‘figure of eight’ pattern. As the disease progresses, scarring occurs with loss of the labia minora, which become fused to the labia majora. The clitoral hood can seal over and the clitoris may be buried (Fig. 57.7). Introital narrowing can lead to difficulties with intercourse and dyspareunia if the skin splits. The vagina is not involved in lichen sclerosus and this may be a useful distinguishing feature from lichen planus. The only exception to this is where there is a significant vaginal prolapse, where the epithelium becomes keratinized and lichen sclerosus can then develop on this vaginal mucosa. Fig. 57.6 Vulval lichen sclerosus: early established disease showing rubbery oedema of labia minora and clitoral hood and stark whitening extending to perianal skin. Note currently healing fissure at 6 o’clock position. (See also colour plate 57.6) Fig. 57.7 Advanced vulval lichen sclerosis: white sclerotic change, complete burying of the clitoris and total replacement of architecture with ‘plastering’ down and resorption of labia. Gross ecchymoses and narrowing of vaginal introitus. (See also colour plate 57.7) Histology shows a thinned epidermis overlying a homogenized band of collagen in the upper dermis with a lymphocytic inflammatory cell infiltrate underlying this (Fig. 57.8). Fig. 57.8 Histology of lichen sclerosus: an atrophic epidermis is seen over the homogenized band of collagen and, below this, a lymphocytic infiltrate. Haematoxylin and eosin × 40. (See also colour plate 57.8) Squamous cell carcinoma (SCC) is a rare complication of lichen sclerosus, occurring in about 4% of patients [3]. This may present as a small persistent erosion or ulcer, a hyperkeratotic area or fleshy friable papule or nodule (Fig. 57.9). Any suspicious lesions must be biopsied. If vulval intraepithelial neoplasia (VIN) is found in association with lichen sclerosus, it is usually the differentiated form where there is basal atypia but normal maturation of the epidermis. It is less common to have undifferentiated VIN. Fig. 57.9 Lichen sclerosus complicated by squamous carcinoma. Note the classical cigarette‐paper scarring and background whitening. Here the squamous cell carcinoma presents as a fleshy nodule, but persistent erosion should also prompt biopsy. (See also colour plate 57.8) There is good evidence that the treatment for lichen sclerosus, in adults and children, is a super‐potent topical steroid such as clobetasol propionate 0.05% ointment [4,5]. This is applied once daily for a month, on alternate days for the second month and then twice weekly for the third month. Emollients are used as a soap substitute. Ongoing treatment may be required by some patients to maintain control. Patients whose disease is difficult to control or those with any history of VIN or SCC need long‐term follow‐up in specialized clinics. There is no role for the use of topical testosterone. Surgery is only required to treat the scarring complications or if there is neoplastic or pre‐neoplastic change. Topical calcineurin inhibitors are increasingly popular but should not be used first line as there are concerns about their long‐term safety in relation to the development of malignancy. In those with resistant symptoms, it is important to exclude an allergic contact dermatitis to treatment, irritant dermatitis due to urinary incontinence, or an additional problem such as herpes simplex or candidiasis. A proportion of patients will develop vulvodynia after their lichen sclerosus is well controlled. Treatment must be targeted at this rather than increasing the use of topical steroids. Lichen planus (LP) is an inflammatory disorder, which can affect both skin and mucous membranes. The characteristic cutaneous lesions are small purplish papules, which may exhibit a fine lace‐like network over their surface known as Wickham’s striae. These can also be seen on mucosal lesions. The papules commonly occur on flexor surfaces and can koebnerize at sites of trauma. The nails can show pterygium formation and scalp lesions can result in a scarring alopecia. Histology shows irregular acanthosis with a saw‐toothed pattern, basal cell degeneration and a dense band‐like dermal infiltrate of lymphocytes. There is often pigmentary incontinence, which is responsible for the marked hyperpigmentation sometimes seen clinically (Fig. 57.10). Fig. 57.10 Histology of lichen planus showing saw‐toothing of the epidermis, with a dense lymphocytic infiltrate and liquefactive degeneration of the basement membrane. Source: Eduardo Calonje. Reproduced with permission of Eduardo Calonje. (See also colour plate 57.10) The cause is unknown but it is likely that it is a T‐lymphocyte‐mediated inflammatory response to some form of antigenic insult. Lichenoid eruptions can be seen in graft‐versus‐host disease and secondary to medications such as non‐steroidal anti‐inflammatory drugs (NSAIDs). Although there has been interest in the association of hepatitis C infection and LP, there is only evidence for this in certain populations and it is not relevant in northern Europe. There is an association of LP and other autoimmune disorders. There are three major clinical patterns of LP affecting the anogenital skin: erosive, classic and hypertrophic. These may occur in isolation without the presence of disease at other sites. Erosive LP is the commonest form to affect the genital skin [6]. There is a specific subtype of erosive mucosal LP, the vulvovaginal–gingival (VVG) syndrome affecting the vulva, vagina and gingival margins [7], with specific genetic associations. The lacrimal duct, external auditory meatus and oesophagus can also be involved and disease at these sites needs a multidisciplinary approach to management. The vulval lesions mainly affect the inner labia minora and vestibule where erythema and erosions occur (Fig. 57.11). A lacy white edge is seen and this is the best site for a confirmatory biopsy (Fig. 57.12). There may be marked scarring with significant architectural change, which can be impossible to differentiate from lichen sclerosus in some cases. Symptomatically the condition is itchy and painful and dyspareunia is a common feature. If there is vaginal involvement, there may be a blood‐stained discharge and episodes of post‐coital bleeding. It is important to recognize vaginal disease as scarring at this site can result in complete vaginal stenosis. Glazed erythema and erosions occur on the gingivae (Fig. 57.13), buccal mucosa and also the tongue. Fig. 57.11 Erosive lichen planus: there is scarring with loss of the labia minora. Wickham’s striae are seen at the edge of the erosions. (See also colour plate 57.11) Fig. 57.12 The lacy white edge of the eroded area is seen. This is the best site for biopsy. (See also colour plate 57.12) Fig. 57.13 Erosive lichen planus with gingival involvement. Erythema and erosions are seen at the gingival margins. Similar lesions may be seen on the buccal mucosa and tongue. (See also colour plate 57.13) The disease tends to fluctuate with a relapsing and recurring pattern. Papules, very similar to the cutaneous lesions, are found on the vulva (Fig. 57.14) and perianal skin. They may be asymptomatic in over 50% of patients [8]. Wickham’s striae can also be seen associated with the lesions. Flexural hyperpigmentation can be significant even many months after the disease has resolved. Fig. 57.14 Papular lichen planus: typical coalescing flat‐topped papules showing white Wickham’s striae. They are violaceous in colour and are usually also found on the inner wrist and elsewhere. (See also colour plate 57.14) Hypertrophic lesions are less common and mainly affect the perineum and perianal skin. They can become ulcerated and painful. They can be resistant to topical treatment. Squamous cell carcinoma and SCC in situ have been reported in classic and hypertrophic types but not in the VVG syndrome. In studies of patients with vulval malignancy, LP has been seen in the surrounding epithelium [9]. The main treatment is a super‐potent topical steroid ointment. In general, clobetasol propionate 0.05% ointment is applied in a similar regimen to that used for lichen sclerosus for 3 months, then reducing to be used as needed. Bland emollients can be helpful as a soap substitute. Petroleum jelly used as a barrier will help symptomatically. For vaginal disease, some of the foam preparations used in inflammatory bowel disease can be useful, for example hydrocortisone acetate. This is inserted via the applicator into the vagina at night. Dilators may also be required to keep the vagina patent. There is some evidence for the use of the topical calcineurin modulators tacrolimus and pimecrolimus in the treatment of LP. There are small case series that support their use but there is also concern with long‐term safety, particularly with regard to the development of malignancy. It is therefore recommended that they are not used as first‐line treatment but for short periods in those who do not respond to potent topical steroids. They are not tolerated well on the anogenital skin, which also limits their use. Several systemic treatments have been used in LP but no controlled trials exist [10].
Benign Diseases of the Vulva
History‐taking
Examination
General view
Specific areas to be inspected
Normal variants
Angiokeratomas
Hart’s line
Vestibular papillae
Fordyce spots
Normal physiological changes
Childhood
Puberty
Pregnancy
Post menopause
Investigations
Biopsy
Microbiological investigation
Patch testing
Inflammatory diseases of the vulva
Lichen sclerosus
Aetiology
Clinical features
Lichen sclerosus and malignancy
Treatment
Lichen planus
Aetiology
Erosive lichen planus
Classic lichen planus
Hypertrophic lichen planus
Lichen planus and malignancy
Management
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