Menopause International > Volume 14, Number 4 > Pp. 169-172

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Reviews

Postmenopausal vulval disease

Ann Olsson ^* , Priya Selva-Nayagam ^* and Martin K Oehler 

^* Vulval Disorders Clinic, Department of Gynaecology
Department of Gynaecological Oncology, Royal Adelaide Hospital, Adelaide, Australia

Correspondence: Ann Olsson MBBS FRANZCOG, Vulval Disorders Clinic, Department of Gynaecology, Royal Adelaide Hospital, Adelaide 5000, Australia. Email: annolsson{at}chariot.net.au

	Abstract

Top Abstract Introduction Lichen sclerosus Lichen planus Vulval dermatitis Psoriasis Chronic candidiasis Vulval intraepithelial neoplasia Paget's disease Conclusion Competing interests References

 
Vulval disease in the postmenopausal age group is relatively common. Some vulval conditions such as lichen sclerosus are more prevalent in the postmenopausal years. Often more than one condition is present at the same time. Accurate diagnosis is essential for effective treatment. The risk of progression to malignancy associated with some of these diseases dictates long-term surveillance.

Key Words: Dermatitis • intraepithelial neoplasia • lichen sclerosus • vulva

	Introduction

Top Abstract Introduction Lichen sclerosus Lichen planus Vulval dermatitis Psoriasis Chronic candidiasis Vulval intraepithelial neoplasia Paget's disease Conclusion Competing interests References

 
Vulval disease can affect any age group. Many women do not present in primary care because of embarrassment. In addition, some general practitioners are unfamiliar with the diagnosis and treatment of some vulval diseases. It is, therefore, difficult to ascertain the actual prevalence of these disorders.

Some conditions have a peak incidence in the postmenopausal years, whereas others are less common at this time in life. Often there is more than one disease process active at the same time in an individual patient. Patients with vulval disorders are most easily managed in a multidisciplinary clinic with the expertise of both gynaecologists and dermatologists. The opinion of a histopathologist with an interest in this area is vital. Consultation with a psychologist or an infectious diseases physician is often required.

The scope of this paper is to discuss those vulval disorders, excluding vulval cancer, more frequently encountered in the postmenopausal woman.

	Lichen sclerosus

Top Abstract Introduction Lichen sclerosus Lichen planus Vulval dermatitis Psoriasis Chronic candidiasis Vulval intraepithelial neoplasia Paget's disease Conclusion Competing interests References

 
Lichen sclerosus (LS) is an inflammatory dermatosis, which has a predilection for the vulval and perianal skin in women. The cause of LS is unknown although there is an association with autoimmune diseases.¹ LS is a common condition presenting to vulval clinics. The true prevalence is unknown.² There are two peak ages of presentation of LS; first in prepubertal girls and secondly in postmenopausal women.

Most women will complain of itching but sometimes the presentation is one of soreness, burning and/or pain without itching. A number of women will be asymptomatic. Clinical features include pallor and atrophy. A figure of eight distribution around the vulva and anus is often described. Erosions, fissures, hyperkeratosis and ecchymoses may also be present. Advanced cases present with distortion of the vulval anatomy with fusion of the labia, burying of the clitoris and narrowing of the introitus.

Diagnosis is either clinical or by biopsy. It is extremely useful to establish the diagnosis by biopsy early in the presentation, as the clinical features may change over time. Consideration should be given to investigating a patient with LS for other autoimmune conditions, such as thyroid disease.^1,3 Recently, an association with psoriasis has been suggested.⁴

The differential diagnosis includes lichen planus (LP), psoriasis and vitiligo. Vitiligo is distinguished from LS by the presence of normal texture to the skin and the lack of inflammatory or secondary changes.

Complications of LS include scarring and malignant potential. The risk of developing a squamous cell carcinoma in LS-affected vulval skin is reported as 5% although this may be an over-estimate.² This risk of malignant change subjects the LS patient to lifetime follow-up; hence the importance of establishing the exact diagnosis by early biopsy.

The mainstay of treatment is topical application of the ultra-potent corticosteroid ointment clobetasol propionate. The guidelines for the management of LS prepared for the British Association of Dermatologists¹ have recommended a regimen of application of clobetasol propionate nocte for four weeks, followed by alternate night application for four weeks with twice weekly application for the third month. If the patient's symptoms flare during this regimen, then a return to the most effective frequency of application is recommended. Review at the three-month point should occur. The ointment is then used as required for symptom control. A 30 g tube should last for 6–12 months.

It should be noted that clobetasol propionate is not commercially available in some countries, such as Australia, except if it is produced by a compounding pharmacy. In Australia, the recommended agent is betamethasone dipropionate, optimized propylene glycol vehicle, as an ointment.

Calcineurin inhibitors are currently under investigation as an alternative to topical steroids in the management of LS.^5,6 Concern has been expressed about this form of treatment as there have been reports of the development of cancers and lymphomas in patients using these preparations.⁷

The diagnosis of LS in women subjects them to ongoing surveillance because of the small risk of progression to vulval cancer. As LS is a common condition, this surveillance is becoming a burden on the specialized vulval clinics. Suggested guidelines have been developed for the follow-up of women with vulval LS in specialist clinics.² These guidelines recommend specialist clinic follow-up for those women with LS who have poor symptom control, i.e. women requiring potent topical steroid application three or more times a week or greater than 30 g/six months; women who have been previously treated for vulval intraepithelial neoplasia (VIN) and/or squamous cell carcinoma of the vulva; women with clinical evidence of localized skin thickening/hyperkeratosis; and finally women whose biopsies are reported as ‘concerning’ by the pathologist, where a definite diagnosis of differentiated VIN cannot be made.

	Lichen planus

Top Abstract Introduction Lichen sclerosus Lichen planus Vulval dermatitis Psoriasis Chronic candidiasis Vulval intraepithelial neoplasia Paget's disease Conclusion Competing interests References

 
LP is an inflammatory dermatosis, which is much less common than LS. LP may affect the vulva and vagina, unlike LS which is confined to the vulva. It can also involve the scalp, oral mucosa, skin and nails.

The cause of LP is unknown but it is thought to have an immunological basis.⁸ The prevalence of LP is unknown. It has a peak incidence between 30 and 60 years of age.⁸

The most common presentation is a complaint of soreness. Other clinical manifestations include itching, burning and dyspareunia. A yellow vaginal discharge may be present if the vagina is involved in the inflammatory process.

Three types of LP affecting the vulva have been described: classical, hypertrophic and erosive.⁹ The classical type includes small papules, a violaceous hue and sometimes a reticulate lace pattern, as seen on the buccal mucosa. Postinflammatory pigment changes may be a feature. Erosive LP is the most common variant seen on the vulva and in the vagina. Erosions appear as deep erythema and are common on the posterior vulval vestibule and labia minora.¹⁰ Architectural distortion of the vulva can occur. In the vagina, the inflammatory process can lead to scarring and stenosis. Hyperkeratotic lesions, consisting of firm white papules or plaques with irregular borders and a thickened irregular surface, are less common.¹⁰

The natural history of LP is usually one of remission with time. Erosive LP of the vulva, however, is usually chronic and persistent.⁸ The diagnosis of LP is usually made clinically. Histology may show non-specific inflammatory changes only;⁸ however, the presence of basal layer vacuolar change with or without a band-like infiltrate is helpful.¹⁰ The differential diagnosis includes LS, bullous disorders, erythema multiforme, fixed drug eruptions, VIN and plasma cell vulvitis.^8–10 It should be noted that LS does not affect the vagina. It is also helpful to look for extragenital LP lesions. Progression to malignancy has been reported but is rare.^9,10

Treatment of LP is the application of topical steroid ointments. Erosive disease requires a potent or ultra-potent steroid. For those women with vaginal disease, steroid preparations are inserted into the vagina via an applicator. There are no formulations specifically designed for the vagina and therefore products available for rectal use are often employed.¹⁰ The use of petroleum jelly as a barrier agent will aid in symptomatic relief. Calcineurin inhibitors are being trialled as second-line therapy in the management of this condition.^11,12 Vulvovaginal LP is a difficult relapsing and remitting condition, which requires control of distressing symptoms. Long-term follow-up is necessary because of the rare risk of squamous cell carcinoma.

	Vulval dermatitis

Top Abstract Introduction Lichen sclerosus Lichen planus Vulval dermatitis Psoriasis Chronic candidiasis Vulval intraepithelial neoplasia Paget's disease Conclusion Competing interests References

 
Dermatitis affecting the vulva may occur at any age and is common. It may complicate the presentation of other dermatoses. The tendency of women to self-medicate with preparations available over the counter in pharmacies and supermarkets exacerbates this problem.

Contact dermatitis is the main type of dermatitis seen and may be irritant or allergic in origin. Estrogen-deficient patients are particularly prone to irritant contact dermatitis.¹³

Itch is the predominant symptom although burning and pain may be described if fissures supervene. Thickening and whitening of the skin (lichenification) arise with longstanding disease. It is sometimes difficult to distinguish clinically a case of longstanding vulval dermatitis (lichen simplex chronicus) from LS. A history of atopy and/or dermatitis or eczema at other sites may assist in the diagnosis. There may also be a family history of atopy.

It is important to document all topical applications that are currently in use as well as all those that have been administered in the past. Common irritants include soaps, antiseptics, disinfectants, tea tree oil and a multitude of creams including those applied for candidiasis.^8,14 Overzealous cleansing of the vulval skin, and urinary and faecal incontinence are also precipitants for an irritant dermatitis of the vulva. The use of sanitary and incontinence pads add to the problem. Potential allergens include topical anaesthetics and antibiotics, topical antifungal agents, perfumes and preservatives.^13,14 Corticosteroid preparations may also cause an allergic contact dermatitis.^8,13 A vulval biopsy may be required to clarify the diagnosis.

The first line of management is to remove all irritants and potential allergens from use. Soap substitutes such as aqueous creams can be employed. These will also act as emollients for the vulval skin.⁸ The addition of a barrier cream, such as petrolatum jelly, is also useful to protect the skin.¹³ Advice regarding the avoidance of over-washing is essential and steps should be instituted to manage any urinary and faecal incontinence if present.

Topical steroid ointments are used to reduce inflammation and to stop the itch–scratch cycle.¹³ Potent steroids are initially employed for 7–10 days to manage cases of lichen simplex chronicus. The potency can then be reduced to hydrocortisone 1% to provide maintenance for two to three months.^14,15

Superadded candidiasis should be treated with an oral antifungal agent, such as fluconazole. Repeated doses may be required for recurrent cases. Patch testing is frequently undertaken to identify those allergens involved.

	Psoriasis

Top Abstract Introduction Lichen sclerosus Lichen planus Vulval dermatitis Psoriasis Chronic candidiasis Vulval intraepithelial neoplasia Paget's disease Conclusion Competing interests References

 
Psoriasis is a common cause of vulvar itch in both pre- and postmenopausal women.¹⁴ The rash has a well-defined border. Useful clues to this diagnosis are the presence of psoriasis elsewhere and a family history. In addition to topical steroid ointments, weak tar preparations and calcipotriol ointment can be tried in these patients.¹⁵

	Chronic candidiasis

Top Abstract Introduction Lichen sclerosus Lichen planus Vulval dermatitis Psoriasis Chronic candidiasis Vulval intraepithelial neoplasia Paget's disease Conclusion Competing interests References

 
This is usually only seen in the postmenopausal setting in women taking estrogen replacement, diabetics or as a secondary infection on an underlying dermatosis.¹⁴ These patients usually require oral treatment and correction of the underlying predisposing condition.

	Vulval intraepithelial neoplasia

Top Abstract Introduction Lichen sclerosus Lichen planus Vulval dermatitis Psoriasis Chronic candidiasis Vulval intraepithelial neoplasia Paget's disease Conclusion Competing interests References

 
VIN has been considered a disease of mid-late life in women. More recently, an increasing number of young women are being diagnosed with human papilloma virus (HPV)-related VIN.

The classification of VIN was modified in 2004.¹⁶ The term VIN 1 is no longer used. The term VIN applies to histologically high-grade squamous lesions (previously VIN 2, VIN 3 and differentiated VIN 3).

VIN is categorized into usual type, differentiated type or unclassified type. VIN, usual type is the most common type and is generally associated with high-risk HPV types. It is further categorized into: warty type, basaloid type or mixed (warty/basaloid) type. VIN, differentiated type is less common and is generally not associated with HPV. It is seen primarily in older women. In some cases it is associated with LS. It is often present in association with keratinizing squamous cell carcinomas.¹⁶

VIN is heterogeneous in its clinical features and behaviour.¹⁷ About one-third to two-thirds of cases may be asymptomatic.^15,17 Symptoms can include itching, burning, pain and the presence of a vulval lump. Examination will usually reveal a macroscopically visible lesion that is often raised and may be red or white in colour. Pigmented lesions also occur. The disease may be unifocal or multifocal.

Spontaneous regression of VIN has been observed, but may be confined to the younger age group (less than 30 years of age) with multifocal pigmented (probably HPV-related) disease.¹⁸ Progression to squamous cell carcinoma is well documented. Surgically treated VIN has a high rate of recurrence. Untreated VIN lesions in women over 30 years of age have an appreciable invasive potential.¹⁷

The mainstay of treatment is conservative surgical excision of VIN lesions, particularly in the older women. Thorough histopathological examination is essential to detect any occult cancer.

Topical imiquimod 5% cream is increasingly used to treat VIN and appears to be an effective option.¹⁹ Side-effects include local irritation, burning and erythema. Imiquimod's effectiveness may be limited to HPV-related disease.¹⁹ Laser ablation has also been employed to treat HPV-related VIN.

There are no guidelines for the follow-up of women treated for VIN. Jones et al.¹⁷ recommend six month reviews for two years after the last VIN treatment and at least annual review thereafter. It has recently been found that VIN related to LS without squamous cell carcinoma is undifferentiated in type.²⁰ This finding has the potential to alter the treatment rationale of patients with VIN in the older age group. It may be feasible to consider more conservative treatments, such as imiquimod cream, for these older women rather than local excision, if these findings are confirmed.

	Paget's disease

Top Abstract Introduction Lichen sclerosus Lichen planus Vulval dermatitis Psoriasis Chronic candidiasis Vulval intraepithelial neoplasia Paget's disease Conclusion Competing interests References

 
Paget's disease of the vulva is a rare intraepithelial neoplasm, which is most commonly seen in postmenopausal women. In about 10–20% of cases, it is associated with an underlying adenocarcinoma of the vulva or adjacent organs.²¹

The most common early symptom is pruritus and associated moderately well-demarcated erythematous patches with white hyperkeratotic areas. In more advanced disease, the lesions become more plaque-like and desquamative. Paget's disease most commonly occurs on the labia majora but may also involve the labia minora, clitoral area, mons pubis, perineum, thigh and/or buttocks.

A delay in diagnosis is not uncommon as it has similarities with eczema but fails to clear up with topical steroid creams. The diagnosis is made by biopsy. Histopathology is distinctive and characterized by clusters of Paget cells. When vulval Paget's disease is diagnosed, potential associated neoplastic disease of the breast, genitourinary tract and gastrointestinal tract should be excluded.²² Periurethral and perianal lesions may indicate secondary involvement or continuity from the primary site.

Treatment of vulval Paget's disease is by surgical excision. Clear margins are very difficult to achieve as the involved area is usually much greater than the visible lesion. Furthermore, multicentric foci occur in macroscopically normal epithelium.²³ Intraoperative frozen section evaluation of the margins has not been shown to reduce the recurrence rate, which is about 30%.^21,24 Positive margins in the final pathology do not have to be re-excised immediately, but long-term monitoring is required.

Further wide local excision is indicated for recurrent disease that can be repetitive and extensive. The use of non-excisional treatments, such as laser, radiotherapy, photodynamic therapy and 5-fluorouracil, can also be considered in recurrent Paget's disease if invasion has been excluded.^25,26 Furthermore, successful treatment of Paget's disease with topical imiquimod has been reported and warrants further investigation.²⁷

The prognosis, in terms of survival, for patients with Paget's disease of the vulva without associated malignancy, is very good. Occasionally, however, the intraepithelial lesion gives rise to an invasive adenocarcinoma with potential lymphatic metastasis requiring radical surgery and possible lymph node dissection like other vulval cancers.

	Conclusion

Top Abstract Introduction Lichen sclerosus Lichen planus Vulval dermatitis Psoriasis Chronic candidiasis Vulval intraepithelial neoplasia Paget's disease Conclusion Competing interests References

 
Women in their postmenopausal years may well be suffering from one or more vulval conditions. Sometimes they are asymptomatic but quite often women will not voluntarily admit to their symptomatology. Some of these diseases carry a risk of progression to malignancy. It is, therefore, important that practitioners caring for these women make every attempt to detect and treat these diseases. Further research is required to elucidate the true prevalence and malignant potential of these conditions as well as to determine appropriate treatment modalities.

	Competing interests

Top Abstract Introduction Lichen sclerosus Lichen planus Vulval dermatitis Psoriasis Chronic candidiasis Vulval intraepithelial neoplasia Paget's disease Conclusion Competing interests References

 
None declared.

Accepted: July 8, 2008.

	References

Top Abstract Introduction Lichen sclerosus Lichen planus Vulval dermatitis Psoriasis Chronic candidiasis Vulval intraepithelial neoplasia Paget's disease Conclusion Competing interests References

 

1. Neill SM, Tatnall FM, Cox NH. British Association of Dermatologists. Guidelines for the management of lichen sclerosus. Br J Dermatol 2002;147:640–9[Medline]
2. Jones RW, Scurry J, Neill S, MacLean AB. Guidelines for the follow-up of women with vulvar lichen sclerosus in specialist clinics. Am J Obstet Gynecol 2008;198:496.e1–3
3. Birenbaum DL, Young RC. High prevalence of thyroid disease in patients with lichen sclerosus. J Reprod Med 2007;52:28–30[Medline]
4. Simpkin S, Oakley A. Clinical review of 202 patients with vulval lichen sclerosus: a possible association with psoriasis. Australas J Dermatol 2007;48:28–31[Medline]
5. Oskay T, Sezer HK, Genc C, Kutluay L. Pimecrolimus 1% cream in the treatment of vulvar lichen sclerosus in postmenopausal women. Int J Dermatol 2007;46:527–32[Medline]
6. Hengge UR, Krause W, Hofmann H, et al. Multicentre, phase II trial on the safety and efficacy of topical tacrolimus ointment for the treatment of lichen sclerosus. Br J Dermatol 2006;155:1021–8[Medline]
7. Lewis FM, Neill SM. Safety of calcineurin inhibitors in the management of lichen sclerosus. Br J Dermatol 2007;156:1389–90[Medline]
8. Ball SB, Wojnarowska F. Vulvar dermatoses: lichen sclerosus, lichen planus, and vulval dermatitis/lichen simplex chronicus. Semin Cutan Med Surg 1998;17:182–8[Medline]
9. Lewis FM. Vulval lichen planus. Br J Dermatol 1998;138:569–75[Medline]
10. Moyal-Barracco M, Edwards L. Diagnosis and therapy of anogenital lichen planus. Dermatol Ther 2004;17:38–46[Medline]
11. Kirtschig G, Van Der Meulen AJ, Ion Lipan JW, Stoof TJ. Successful treatment of erosive vulvovaginal lichen planus with topical tacrolimus. Br J Dermatol 2002;147:625–6[Medline]
12. Lonsdale-Eccles AA, Velangi S. Topical pimecrolimus in the treatment of genital lichen planus: a prospective case series. Br J Dermatol 2005;153:390–4[Medline]
13. Margesson LJ. Contact dermatitis of the vulva. Dermatol Ther 2004;17:20–2[Medline]
14. Welsh B, Howard A, Cook K. Vulval itch. Aust Fam Physician 2004;33:505–10[Medline]
15. Welsh BM, Berzins KN, Cook KA, Fairley CK. Management of common vulval conditions. Med J Aust 2003;178:391–5[Medline]
16. Sideri M, Jones RW, Wilkinson E, et al. Squamous vulvar intraepithelial neoplasia: 2004 modified terminology, ISSVD Vulvar Oncology Subcommittee. J Reprod Med 2005;50:807–10[Medline]
17. Jones RW, Rowan DM, Stewart AW. Vulvar intraepithelial neoplasia: aspects of the natural history and outcome in 405 women. Obstet Gynecol 2005;106:1319–26[Medline]
18. Jones RW, Rowan DM. Spontaneous regression of vulvar intraepithelial neoplasia 2-3. Obstet Gynecol 2000;96:470–2[Medline]
19. van Seters M, van Beurden M, ten Kate FJ, et al. Treatment of vulvar intraepithelial neoplasia with topical imiquimod. N Engl J Med 2008;358:1465–73[Abstract/Free Full Text]
20. van Seters M, ten Kate FJ, van Beurden M, et al. In the absence of (early) invasive carcinoma, vulvar intraepithelial neoplasia associated with lichen sclerosus is mainly of undifferentiated type: new insights in histology and aetiology. J Clin Pathol 2007;60:504–9[Abstract/Free Full Text]
21. Fanning J, Lambert HC, Hale TM, Morris PC, Schuerch C. Paget's disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision. Am J Obstet Gynecol 1999;180 (Pt 1):24–7[Medline]
22. ACOG Practice Bulletin No. 93: diagnosis and management of vulvar skin disorders. Obstet Gynecol 2008;111:1243–53[Medline]
23. Gunn RA, Gallager HS. Vulvar Paget's disease: a topographic study. Cancer 1980;46:590–4[Medline]
24. Black D, Tornos C, Soslow RA, Awtrey CS, Barakat RR, Chi DS. The outcomes of patients with positive margins after excision for intraepithelial Paget's disease of the vulva. Gynecol Oncol 2007;104:547–50 (Epub 24 October 2006)[Medline]
25. Moreno-Arias GA, Conill C, Sola-Casas MA, Mascaro-Galy JM, Grimalt R. Radiotherapy for in situ extramammary Paget disease of the vulva. J Dermatol Treat 2003;14:119–23
26. Shieh S, Dee AS, Cheney RT, Frawley NP, Zeitouni NC, Oseroff AR. Photodynamic therapy for the treatment of extramammary Paget's disease. Br J Dermatol 2002;146:1000–5[Medline]
27. Hatch KD, Davis JR. Complete resolution of Paget disease of the vulva with imiquimod cream. J Low Genit Tract Dis 2008;12:90–4

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