Vulval examination

Ecchymosis (subcutaneous purpura) seen in lichen sclerosus

Scabies

Squamous cell carcinoma

There is a high chance of symptom response following the 3­month course, but lichen sclerosus remains a chronic skin disease and the symptoms are likely to recur if the treatment is stopped. Regular use of topical steroids in the long term has been shown to be safe and may reduce the underlying small risk of vulval cancer (see ‘The use of topical steroids') (21).

Patients may develop symptomatic skin fissuring at the posterior fourchette. Treatment should involve digital massage of topical

Table 50.3 Summary of the clinical features and treatment of specific skin diseases

Figure 50.5 Advanced vulval lichen sclerosus. Whiteness, loss of anatomy, ecchymosis seen on the left labia, and scarring over the clitoral hood.

steroid into the fissure on a daily basis. The use of vaginal dilators and a good lubricant should be encouraged to help the scar re­forming and help desensitize the area. If these measures do not help then consideration should be given to the use of surgical division or refashioning of the fissure or skin bridge.

There is a small risk of cancer development in lichen sclerosus (with findings of typical eczema elsewhere. Patients rarely complain of vulval eczema when there is severe eczema elsewhere. However, in a patient who complains of vulval itching and who has a his­tory of atopy, it is worth considering as a diagnosis. Vulval atopic eczema is often seen clinically as intralabial, perianal, and natal cleft erythema. The erythema is often mild. Signs of atopic ec­zema elsewhere include poorly defined, symmetrical, scaly ery­thematous areas on the skin creases (especially the antecubital fossae and behind the knees). Small fissures and erosions may be present. The skin is often noticeably dry. Severe eczema is more widespread over the trunk and limbs. Atopic vulvitis is the com­monest cause of vulval itch in children.

Contact dermatitis may be either ‘irritant' or ‘allergic contact'. Irritant contact dermatitis is particularly common and may be trig­gered by soaps, perfumes, medicaments, urine, faeces, and sweat. The barrier function of the skin becomes impaired by local irritants and can subsequently be made worse by continued application of the product. Clinical signs of vulval irritant dermatitis include poorly defined intralabial erythema that occurs anywhere where the irri­tant has been present. Small fissures and erosions may be present and lichenification may occur in longstanding disease (15). All of these clinical features are demonstrated in Figure 50.2. It is common that fissures become secondarily infected with skin pathogens or Candida. It is important to realize this when treatment strategies are implemented. Allergic contact dermatitis is less common and around one-fifth of patients with vulval skin conditions having a relevant positive patch test result (15). It can be difficult to distinguish from irritant contact dermatitis clinically, but affected skin usually ex­tends outside of the genital area in ‘non-contact' areas. This is be­cause it is an immune-mediated hypersensitivity reaction. The only way to confidently diagnose allergic contact dermatitis is through patch testing (see ‘Investigations').

Vulval seborrhoeic eczema is difficult to distinguish from psor­iasis. It will often manifest as glazed skin in the interlabial sulci bilaterally. Fine scale and erythema at other affected body sites such as the nasolabial folds, scalp, and eyebrows can aid the diagnosis (26).

Treatment principles for inflammatory vulval skin conditions

There are no core clinical outcome measures for vulval skin disease (24) and suggested clinical outcomes include:

• a reduction in symptoms (e.g. less itch, pain, and fewer flare- ups of symptoms)

• an improvement in function (e.g. sexual function, mobility, and normalizing bladder and bowel function)

• increased confidence in self-management (e.g. management of flare-ups and self-examination).

General principles

Initial principles of management are the same for all vulval skin con­ditions and a holistic approach is required. Good education, support, and counselling are important with extra time given to address the disease process, discussing general vulval care measures, and man­aging patient expectations (1). It is useful to provide information leaflets, direct patients to relevant patient-oriented websites, and write down instructions for applying topical agents (see ‘Additional resources' for sources of patient information). The use of a mirror or model in the clinic setting is helpful to show patients where to apply their topical treatments.

Correct barrier function

The goal of treatment is to correct barrier function and reduce in­flammation. For washing, soap and other routine cleaning agents (e.g. wipes) should be avoided, as they are likely to act as irritants and sensitizing allergens. Irritation from urinary and faecal incon­tinence need to be addressed as these are a common cause of vulval inflammation and make underlying skin pathology worse. ‘Soap substitution' with a bland cream or ointment-based emollient is best for cleansing. The same agent can then be used as an emollient to both provide a barrier to the site and sooth inflamed skin. There is no preferred emollient to use and some can cause irritation. Emollient creams (not ointments) can be placed in the fridge. Patients find this soothing and lowering skin temperature is thought to reduce itch through central inhibitory pathways (27).

The use of topical steroids

Inflammation reduction associated with skin disease (such as in li­chen planus, lichen sclerosus, and eczema) is achieved by the use of topical steroids. Topical steroids are often ineffectively used in the vulval area due to concerns from patients and non-specialists about side effects, particularly skin or mucosal atrophy. It is important therefore to use the correct strength of steroid for the necessary length of time on the appropriate body site. Mucosal surfaces such as the vulval vestibule are remarkably resistant to steroid atrophy. In contrast, keratinized surfaces such as the labiocrural folds, peri­neum, perianal area, and thighs can develop skin thinning and striae (stretch marks) with inappropriate use with potent topical steroids (1). Overuse of topical steroids appears as thinned skin which ap­pears redder and is reversible in the early stages. In the later stages, permanent telangiectasia and striae can develop. Topical calcineurin inhibitors in the vulval region reduce inflammation and do not cause skin atrophy. However, their role is not fully understood and there is a theoretical risk of long-term localized immunosuppression from these agents causing skin cancers (28).

Topical steroids should be used once a day. There is no evidence to suggest that twice-daily application is superior, although twice- daily application has greater potential to cause side effects (29). Ointments are preferable to creams as they contain fewer constitu­ents and therefore have a lower chance of causing irritation/con- tact allergy. Once control of inflammation and symptoms has been achieved, topical steroids should be reduced to the minimum fre­quency required to maintain remission. The concept of ‘weekend therapy', that is, applying topical steroids on two consecutive days per week, is effective in atopic eczema patients (30) and can be ex­trapolated to chronic vulval diseases such as lichen sclerosus and lichen planus where long-t erm maintenance therapy is required. A patient with these conditions will use approximately 30-60 g of topical steroid per year as maintenance therapy (12). Topical ster­oids should only be used on affected areas to prevent side effects in adjacent skin and the use of a mirror is often helpful to aid applica­tion in the correct areas.

Failure to respond to treatment

If a patient fails to respond to appropriate treatment the following should be considered:

1. Poor adherence to prescribed treatment regimen: ‘steroid phobia' is a well-recognized problem when treating skin con­ditions. Many healthcare professionals, including pharmacists, will compound the issue by advising the patient to use sparingly and recommending not to use on ‘sensitive areas'. This cautious approach can be detrimental to the patient's treatment plan. The patient should be advised to apply the topical steroid in terms of the finger-tip unit (A finger-tip is from the very end of the finger to the first crease in the finger. It does not mean a blob on the fingertip). The number of finger-tip units required is usually one to two but is specifically tailored to the patient depending upon surface area affected by the condition.

2. Inaccurate placement of topical steroid: the patient may be ap­plying the topical treatment to an unaffected area. This is es­pecially common if the patient is elderly and unable to use a mirror to see what they are doing. In clinic the exact location of application should be explained, Diagrams, photographs, or models can be used as an aid.

3. Continued exposure to irritants: urine or faeces, external prod­ucts such as wipes or non-prescribed topical treatments, and over-washing with water can all contribute towards irritation and ongoing symptoms.

4. An incorrect diagnosis. If adherence and skin care practices are adequate, the diagnosis may be incorrect. An allergic contact dermatitis to topical treatments may have occurred (see ‘Patch testing') or there may be premalignant or malignant change in the affected area. If there is any concern, a biopsy should be taken.

The multidisciplinary team

While many patients can respond well to topical treatments, there are many women who have complex needs that can be over­looked. These patients do not tend to follow any agreed clinical pathway, have unmet needs, and require adequate assessment and management (31).

These women require the care of a gynaecologist with a special interest in the management of vulval disease within the context of an appropriate multidisciplinary vulval service. Indications for multi­disciplinary input include:

• ongoing symptoms despite appropriate use of topical steroids

• for review of pathology results by a gynaecological pathologist

• associated vulval intraepithelial neoplasia (usual or differentiated type)

• need for patch testing

• for consideration of surgery

• for resolution of ongoing sexual problems.

Other members of a vulval service might include dermatology, genitourinary medicine, physiotherapy, pain management, psycho- sexual therapy, pathology, and urogynaecology.

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