Vulval dermatoses can be distressing for patients, with negative associations with sexually transmitted infections, cancer and cultural taboo all leading to a delay in presentation.[1] Furthermore, diagnoses of such dermatoses can be challenging for the general practitioner, as the changing anatomy of the vulva due to age and subtle differences between presenting symptoms.[2]

The aim of this review is to discuss the clinical assessment for vulval disease, before introducing the common non-cancerous pruritic skin conditions affecting the vulva in adults (see Box 1), how to manage these dermatoses in the community, and when to refer to secondary care (see Figure 1).[3]

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Clinical assessment

Obtaining a full history is fundamental in making a diagnosis. The patient should be asked about itching, pain and any change in vaginal discharge, with a description of the location, timing, duration, triggers and the impact on function.[2] Their cleaning regime of the vulval area and a complete sexual history that incorporates sexual practices, sexually transmitted infections, genital surgery as well as any urinary or faecal incontinence should be obtained.[4]

Additional probing on any systemic illnesses with a complete dermatological history is needed, comprising atopy and autoimmune disease and any familial link with such diseases should be sought.[4]

Furthermore, the psychosexual impact including fear or anxiety with sexual activity, vaginismus, loss of libido, relationship of symptoms to stress and depression should be asked.[3]

Vulval care with products such as soaps, lubricants and sanitary pads, as well as prescribed treatments and those purchased over the counter need to be elicited, with frequency and length of use, including any failed treatments. The patient’s exercise regime should also be reviewed to see if sweat or type of clothing may be a trigger.[4]

A conventional physical examination needs to be carried out, including the eyes, skin, mouth, thyroid and nails for pathology not limited to the vulva. For example, looking for dermatitis or psoriatic plaques of the skin, pitting or onycholysis of the nails in psoriasis, or papules in the mouth seen in lichen planus.[2] Examination of the vulva, in the presence of a chaperone, needs to identify ulceration, erythema, atrophy, fissures, pigmentation, lichenification or scarring.[2] The vaginal walls should also be examined with a speculum, looking for ulceration, erythema, petechiae or synechiae.[2] The clitoral hood should be visualised for fusion and tightening of the introitus from adhesions.[2]

Vulval dermatitis

Vulval dermatitis is inflammation of the skin secondary to an external agent, with a reported incidence of up to 30% in vulval clinic.[5]

This group can be further differentiated into eczematic and contact dermatitis described below, although the key symptoms are vulval pruritis and soreness.[6] Iron deficiency anaemia is found in 20% cases, with theories for this including changes in enzyme function and neurological dysfunction.[5, 7]

Eczematic – More commonly seen in patients with an atopy history, with warmth, sweat and rubbing associated triggers.[2] Erythematous patches and chronically thick, lichenified plaques will be seen.

Contact – Occurs secondary to contact with an allergen or irritant. Allergic dermatitis is diagnosed by patch testing with familiar sensitisers including topical antibiotics, antifungals, rubber, nickel and preservatives in products.[8] Irritant dermatitis should be contemplated if the history suggests a substantial hygiene habit, as irritation can occur from constant scrubbing of the vulva or from the products used.[2] Exposure triggers include urine, hygienic products, douches and lubricants.[8]

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Management in the community - General vulval care needs to be advocated (see Box 2)[9] and serum ferritin should be checked to rule out iron deficiency anemia. A moderate topical steroid applied once daily (see Box 3),[3] or potent topical steroid if more severe, for 7-10 days until the symptoms resolve.[10]

When to refer to secondary care - If the dermatitis does not improve with treatment or atypical characteristics or lesions appear, then refer to a multidisciplinary vulval clinic for a biopsy.[11]

Vulval psoriasis

Psoriasis is a chronic skin disease with typical erythematous patches with scaling. Patients may have other signs such as nail changes indicative of this diagnosis. Vulval psoriasis presents with pruritus and a burning sensation, made worse by friction and irritants. Patches are well defined and can affect the natal cleft[11], however, minimal scaling is seen (see Figure 2).

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Management in the community - General vulval care is advocated (see Box 2). All patients should be referred to secondary care for management for an initial assessment.[3] If there is a delay in being seen, a moderate topical steroid can be used, with a potent steroid in severe cases (see Box 3). If a secondary infection is diagnosed then a combined agent such as trimovate should be used.

Specific psoriasis treatment is the same for other parts of the body, with topical coal tar and vitamin D analogues such as talcalcitriol and calcipotriol used alone or in combination with topical steroid.

When to refer to secondary care – As this is a chronic disease with few effective treatments, there should be an initial assessment in secondary care. The same course is recommended if the diagnosis is unclear or a lesion is seen and a biopsy is indicated.[11]

Vulval lichen simplex chronicus Lichen simplex chronicus is a chronic inflammatory dermatoses, with the main vulval symptom being severe pruritus, which may lead to vulval pain and an itch/scratch cycle developing.[12]

The primary cause of pruritis may be due to an irritant, dermatitis or secondary to a systemic or psychological disorder which should be sought.[13]

A focal, chronic eczematous patch is seen, which may then become lichenified (see Figure 3) and may be more evident on the side opposite the dominant hand. Skin excoriation and loss of pubic hair from scratching may be observed.

Management in the community – General vulval care is advocated (see Box 2). A topical steroid once daily is required, with a potent steroid used in severe cases (see Box 3). However, symptoms will relapse until any lichenification resolves, so it is advised to taper down steroid use over three months if present.[11] The skin barrier needs to be recovered and the application of waxy emollients such as a layer of petrolatum jelly can be effective.[14] Amitriptyline or doxepin at a low dose, rather than a sedating antihistamine at night is recommended.[2] If there are significant related anxiety and depressive symptoms, psychological therapies may be beneficial.[12]

When to refer to secondary care – If the diagnosis is unclear, then a biopsy may be indicated to rule out a squamous cell carcinoma or if a lesion is seen. A patch test may be required for suspected contact dermatitis.[11]

Vulval lichen sclerosus

Lichen sclerosus is a chronic inflammatory condition with an autoimmune pathogenesis.[15] It has an estimated prevalence of 3% in older aged females.[16]

Bimodal peaks of incidence of vulval lichen sclerosus are seen in prepubertal and menopausal women, with chief symptoms of pruritis, soreness and dyspareunia.[13] Characteristically, white, pearly papules are seen in a ‘figure of eight’ distribution around the vulva, perineal body and perianal skin, with purpura and risk of hyperkeratosis and erosions [15] (see Figure 4). Due to scratching, fissuring and ulceration may occur. Scarring can occur, leading to fusion of the clitoral hood and the posterior fourchette can complicate leading to sexual dysfunction.[17] The risk of progression to squamous cell carcinoma is under 5% [18].

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Management in the community – Patients should be informed of the small risk of carcinoma, to self-examine regularly and to come for urgent review if any changes occur. An autoimmune thyroid and pernicious anemia screen should be completed if symptoms are suggestive and a vulval skin swab to determine any co-existing infection.[11] diagnosis is unclear, then a biopsy may be indicated to rule out a squamous cell carcinoma or if a lesion is seen. A patch test may be required for suspected contact dermatitis.[11] No optimal treatment regime is known but one advocated is using an ultra-potent steroid daily for one month (see box 3), then alternate days for one month and finally twice a week for a month.[19]

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The minimal topical steroid needed to continue remission ca be used as maintenance, with patients typically using 30-60g of topical steroid per year as maintenance.[20] Patients should be reviewed after three and nine months and then annually if stable and uncomplicated.[11]

When to refer to secondary care - If the diagnosis is unclear, or a lesion is seen or there is a concern about vulval intraepithelial neoplasia or malignancy, then a biopsy is needed. At each review, if any pseudocyst of the clitoris is seen, or if dysaesthesia or psychosexual issues are described, then referral is warranted. At the annual review, if the patient remains symptomatic despite optimal treatment or is using the ultra-potent steroid more than three times a week or more than 30g in six months, or suspicious lesions are seen then refer.[21] Patients should also be seen in a specialist vulval clinic if systemic treatments are indicated, with trials showing positive effects with oral retinoids[22] and topical calcineurin inhibitors.[23]

Vulval lichen planus

Vulval lichen planus is a chronic inflammatory, autoimmune dermatosis, most commonly seen in post-menopausal women.[24] Patients may have co- existing oral lichen planus in a syndrome known as vulvovaginal gingival syndrome.[25] The main symptoms described are pruritus, dyspareunia and vaginal discharge. The key differentiating feature with lichen sclerosus is involvement of the vagina.[18] The incidence of progression to squamous cell carcinoma is around 3%.[26]

Clinical signs are of three subtypes: 

1. Erosive – This is the most common subtype with pain the key symptom. A fine, white, hyperkeratotic line, known as Wickham striae, is seen at the edges of erosions in the vestibular area giving a striking red appearance. As the erosions heal, adhesions and stenosis can occur[28] (see Figure 5).

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2. Classical – Violaceous papules are seen over the anogenital region with a lacy, reticular white Wickham striae superimposed[29] (see Figure 6).

3. Hypertrophic – Thick white, wart like plaques occur which can ulcerate (see Figure 7).

bjfm pruritic fig8

Management in the community – Patients should be informed of the risk of carcinoma, to self-examine regularly and to come for urgent review if any changes occur. An autoimmune thyroid screen should be completed[30] and a vulval skin swab taken to determine any co-existing infection. Insufficient evidence on the optimal regime is available, but an ultra-potent topical steroid (see Box 3), parallel to the management of lichen sclerosus, is supported, as is advice is maintenance and quantity of steroid use.[21] Stable disease should be reviewed annually.

When to refer to secondary care – If the diagnosis is unclear, or if a lesion is seen and there is a concern about vulval intraepithelial neoplasia or malignancy, then a biopsy is needed. Patients should also be seen in a specialist vulval clinic with erosive disease as they require long-term follow up, or if systemic treatments are indicated which include oral prednisolone, ciclosporin or retinoids.[11]

Table 1 summaries this article, describing key points on how to differentiate between the various vulval dermatoses from presentation to management.

Attribution

We would like to thank DermNet New Zealand for their permission to use their Figures 2,3,5,6 and 7 in this publication (licence available from http://creativecommons.org/licenses/by-nc-nd/3.0/nz/). No changes were made to the original images. We also thank the patients for consent to use Figure 4 in this article.

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Mitesh Patel ST2 Academic Clinical Fellow, General Practice, Nottingham City Hospital, Nottingham, Corresponding author email address: mpatel59@doctors.org.uk

David Nunns Consultant Gynaecological Oncologist, Nottingham City Hospital, Nottingham