Vulval symptoms after the menopause – Not all atrophy!

Introduction

The effect of the menopause on the vulva and vagina is well recognized, with thinning and atrophy of the mucosa as estrogen levels fall. Vaginal secretions decrease, therefore reducing lubrication which may lead to some discomfort during intercourse. Other normal changes include a loss of fat in the labia majora, thinner labia minora and a shorter vaginal length. ¹

When a woman presents with vulval or vaginal symptoms around or after the menopause, it is therefore easy to assume that they are related to these physiological changes. However, without a full history and systematic examination, it is equally easy to miss other important diagnoses, where early recognition and appropriate management is vital. This review will discuss the clinical features of the common vulval conditions seen after the menopause, their basic management, and advice on when to refer to a specialized Vulval Clinic.

Lichen sclerosus

Lichen sclerosus (LS) is a common vulval dermatosis which has a predilection for the ano-genital skin. ⁶ It is an inflammatory disorder with specific clinical and histological changes but the precise aetiology is unknown. The major peak in incidence is after the menopause, but there is little evidence to suggest that it is directly related to it, and hormonal treatments do not help in its management. It is estimated to occur in 1 in 30 elderly women. ⁷ There is an association with other auto-immune conditions, predominantly thyroid disease in over 30% patients.^8,9

The usual presenting symptom is itch, which can be severe and may disturb sleep. If fissuring occurs, then soreness and dyspareunia will ensue.

The characteristic features are white atrophic plaques which are usually symmetrical affecting the inner aspects of the labia majora, labia minora and over the clitoral hood (Figure 1). Oedema is sometimes seen and the pathognomonic sign is ecchymosis due to rupture of tiny dermal vessels. Extension to the perianal skin occurs in about 30% of patients. The vagina is never involved in LS apart from the rare situation when a significant prolapse protrudes and the epithelium becomes keratinized. LS can then occur on this prolapsed mucosa.^10,11 OPEN IN VIEWER

Without adequate treatment, lichen sclerosus will progress to cause scarring and anatomical distortion. The labia minora can be resorbed completely, the clitoral hood can seal over the clitoris and if the labia minora then fuse together, this can lead to significant introital narrowing. There may be resultant issues with micturition and sexual function which are important to prevent with adequate treatment. These anatomical changes are not seen with simple post-menopausal atrophy. Cases are reported of labial fusion causing urinary incontinence and recurrent infection are reported ¹² and it is very likely that this was due to a scarring dermatosis, but no mention is made of the histology. While scarring cannot be reversed, it should not progress once treatment is started.

There is a small risk (about 4%) of a squamous cell carcinoma developing on lichen sclerosus ¹³ with those who have the hyperkeratotic and acanthotic type of the disease being at greatest risk. Any resistant areas not responding to treatment should be biopsied with careful clinico-pathological correlation employed to identify the subtle changes of differentiated vulval intra-epithelial neoplasia. This is generally not associated with human papilloma virus, but has a very high likelihood of developing into an SCC, and is often diagnosed in the surrounding epithelium when the tumour is excised.

The first line management of LS is an ultra-potent topical steroid and clobetasol propionate 0.05% ointment is generally used.^14,15 The recommended regime is a three-month induction regime and then as required. Emollients as a soap substitute are also helpful. Patients need follow-up to ensure adequate control of symptoms.

Patients who have atypical disease, or who are apparently resistant to topical steroids should be referred for specialized management. Any patient with a history of differentiated VIN or an SCC with LS should be seen in a Vulval Clinic. ¹⁶

Extra-mammary Paget’s disease

Extra-mammary Paget’s disease (EMPD) is an intra-epithelial adenocarcinoma. The vulva is a common site for this rare disease and most cases are primary, but about 30% may be secondary with epidermal involvement from an underlying carcinoma.^19,20

It typically occurs in women over the age of 50 and can be present for many years before diagnosis, as it may be asymptomatic initially, but eventually becomes pruritic and sore. The lesions are erythematous, sometimes macerated plaques (Figure 3) and the site is important as perianal disease is more likely to be associated with a rectal or colonic malignancy. It can mimic psoriasis or eczema but it is unusual for these to start in the elderly population and they should respond rapidly to appropriate treatment with a topical steroid. If they do not, a biopsy is mandatory. Histologically, there can also be diagnostic difficulties as Bowen’s disease and melanoma can look similar. Immunocytochemical studies will help. Once the diagnosis is confirmed, appropriate investigation to exclude an associated malignancy is needed.^21,22 OPEN IN VIEWER

The management of EMPD is challenging as there is a high rate of recurrence with most treatments. ²³ The ultimate aim is cure but in clinical practice this may not be possible as elderly patients may not be fit enough for extensive surgery and control of symptoms and careful monitoring to detect any early signs of invasion are the goals. Surgery, with wide margins taken outside the clinical extent of disease, frequently shows histological disease at the margins and carries a recurrence rate of over 40%. Even with Mohs micrographic surgery where the margins are controlled, 27% may recur. ²⁴ Other options include photodynamic therapy and radiotherapy. Trastuzumab may have a role if the Paget’s is HER2 positive. ²⁵ The role of imiquimod in the management of EMP is promising and can be very useful in reducing the extent of disease and in some cases clearing it.^26,27 Treatment periods may be prolonged as it can cause a significant inflammatory reaction, so can only be applied once or twice a week.

Vulvodynia

Vulvodyna is defined as ‘vulval discomfort, most often described as burning pain, occurring in the absence of relevant visible findings or a specific, clinically identifiable, neurologic disorder’. ²⁸ It is then subdivided into generalised and localised types and the pain may be spontaneous or provoked. Some patients have a mixed type. The generalised, spontaneous type is most commonly seen in older women. The symptoms are usually described in terms of burning, rawness, discomfort and pain which may radiate to the thighs and anal area. The vulva looks entirely normal and neurological examination is also normal. Investigation is not needed as the diagnosis is clinical based on the symptoms and lack of clinical signs. There is a significant association with other painfull conditions such as fibromayalgia, irritable bladder and migraine. ²⁹

One of the most important aspects of the management is a clear explanation of the condition, acknowledging that it is a neuropathic problem and that the pain is real, even though clinical examination is normal. Tricyclic drugs such as amitriptyline or nortriptyline, in low doses are used for their nerve modulating effects. Gabapentin and pregabilin are alternatives. There is no role for topical steroids as there is good evidence that this is not an inflammatory condition. If the pain is generalised, topical lidocaine, widely used in localised, provoked pain such as vestibulodynia, is not helpful.^30–32 In some cases, referral to a pain specialist is needed for consideration of other treatment modalities.

Conclusion

When assessing a post-menopausal patient with vulval symptoms, it is important to make an accurate diagnosis, and not attribute the problem to the menopause without excluding other disorders. Symptoms can sometimes be similar, for example, discomfort with intercourse may be related to lichen planus. Loss of normal architecture cannot be attributed to post-menopausal atrophy, but may be a sign of lichen sclerosus or lichen planus. In those with continuous symptoms but normal examination, consider vulvodynia. Prompt diagnosis and the correct management will give the patient symptomatic relief and help to prevent future complications.