oPatientPlus articles are written by UK doctors and are based on research evidence, UK and European Guidelines. They are designed for health professionals to use, so you may find the language more technical than the condition leaflets.
The vulva is affected by lack of oestrogen after the menopause. Vulvar pruritis and irritation are common symptoms in a postmenopausal woman. Examination of the vulva should exclude finding ulceration or a mass that may accompany these, as they may also be indicative of infection, inflammation, or malignancy.
When a patient presents with a vulval ulcer the following need to be excluded with culture and/or biopsy with colposcopy:
Sexually transmitted infection:
- Herpes simplex.
- Granuloma inguinale.
- Lymphogranuloma venereum.
- Syphilis (NB: this should not be forgotten as the number of cases in the UK is increasing).
- Invasive disease of the vulva.
- Vulval intraepithelial neoplasia (VIN).
- Paget's disease of the vulva.
Other ulcerative conditions:
- Behçet's disease.
- Disseminated lupus erythematosus.
- Invasive disease of the vulva
- Benign tumours:
Premalignant disease of the vulva
Vulval intraepithelial neoplasia (VIN)
- Nature: this is considered a premalignant state. It can occur by means of cell transformation in already existing vulvar disorders such as lichen sclerosus and squamous cell hyperplasia or it can occur independently.
- Presentation: most patients have pruritus, but some are asymptomatic. The lesions may be white, grey, red or raised.
- Management: biopsy is performed before laser therapy, to make sure that a lesion does not contain invasive cancer. Conventional treatment is wide local excision or laser ablation. Because of the close association of VIN with human papillomavirus infection, lifelong follow-up is required to watch for recurrence.
- Prognosis: spontaneous regression of the disease at all stages has been reported.
Malignant disease of the vulva
Paget's disease of the vulva
- A patient with Paget's disease of the vulva (adenocarcinoma in situ) may present with pruritus and weeping or bleeding of the lesion.
- The lesion appears to have an eczematous or velvet-like surface.
- Wide local excision is recommended, because the margins of Paget's disease often extend beyond what is seen on gross examination.
- This explains the high incidence of recurrence.
- Because of the 20% incidence of underlying adenocarcinoma, the dermis should be removed for accurate diagnosis.
- Thorough evaluation of the cervix, colon, bladder, gallbladder and breasts is necessary when Paget's disease of the vulva is found; there is a 30% incidence of concomitant primary carcinoma in these locations.
Vulval cancer is a very rare disease and, on average, a general practitioner will only see a new case once every 7 years. There should be a high index of suspicion for abnormal lesions on vulva, including 'warts' in the postmenopausal woman.
- Vulval cancer accounts for approximately 5% of all female genital malignancies.
- It occurs in about 1.5 per 100,000 women-years in developed countries but is 2-3 times more frequent in underdeveloped countries.
- With the exception of the rare sarcomas, this cancer appears most frequently in women aged 65-75 years.
- 85% are squamous, less than 5% are melanoma, the second most common type.
- Squamous carcinomas can arise on a background of atrophic changes such as lichen sclerosus, or in hypertrophic epithelium.
- Squamous vulval cancer can have many different growth characteristics.
- It can occur in an area of epithelial neoplasia that develops into a small nodule, which may break down and ulcerate.
- Small, warty or cauliflower-like growths may be seen and confused with condylomata acuminata.
- 75% of all growths are primarily on the labia.
- Long-term pruritus, lumps or masses on the vulva are present in most patients with invasive vulval cancer.
- Staging recommendations are from the International Federation of Gynecology and Obstetrics (FIGO).
- Most are treated with radical vulvectomy, but in advanced stage primary vulval cancer, treatment is tailored to individual patient needs. Combined treatments have been developed, using chemotherapy, radiotherapy and surgery.
- Melanoma should be considered if there are pigmented lesions on the vulva.
- They are suspicious if they are blue-black in color, have a jagged or fuzzy border, are raised or ulcerated, or are larger than approximately 1 cm.
- Melanomas may be misdiagnosed as undifferentiated squamous carcinoma, particularly if they are amelanotic.
- Most melanomas are located on the labia minora or clitoris and prognosis is related to the size of the lesion and the depth of invasion.
Non-neoplastic skin conditions
- Psoriasis is a multifocal disease that may affect vulvar tissue as well as skin of the joints, knees and scalp.
- Seborrhoeic dermatitis, another multifocal disease of the sebaceous glands and commonly affecting the scalp, may affect the labia majora only.
- Tinea cruris begins as raised, sharply demarcated, red lesions on the thighs and can spread to the labia.
- These lesions are best diagnosed by punch biopsy with local anaesthetic.
- When a woman presents with vulval symptoms, a vulval examination should be offered.
- If an unexplained vulval lump is found, an urgent referral should be made.
- Vulval cancer can also present with vulval bleeding due to ulceration. A patient with these features should be referred urgently.
- A patient who presents with pruritus or pain may be reasonably managed with a period of 'treat, watch and wait'.
- This should include active follow-up until symptoms resolve or a diagnosis is confirmed.
- If symptoms persist, the referral may be urgent or non-urgent, depending on the symptoms and the degree of concern about cancer.
- Other paths of referral may include a genitourinary medicine clinic, gynaecology or dermatology, based on appearance and suspicions of the examining GP.
Further reading & references
- Canavan T, Cohen D; Vulvar Cancer. American Family Physician; October 2002.
- Gupta R, Warren T, Wald A; Genital herpes. Lancet. 2007 Dec 22; 370(9605):2127-37.
- Sen P, Barton SE; Genital herpes and its management. BMJ. 2007 May 19;334(7602):1048-52.
- Young F; Syphilis: still with us, so watch out! J Fam Health Care. 2006;16(3):77-81.
- Gynaecological cancer - suspected, Clinical Knowledge Summaries (2005)
- Quinn A; Bartholin Gland Diseases, Medscape, Nov 2012
- Meffert J, Lichen Sclerosus et Atrophicus, Medscape, Mar 2011
- Jones RW, Rowan DM, Stewart AW; Vulvar intraepithelial neoplasia: aspects of the natural history and outcome in 405 women. Obstet Gynecol. 2005 Dec;106(6):1319-26.
- Barhan S, Ezenagu L; Vulvar Problems in Elderly Women. Postgraduate Medicine On-line (Oct 1997).
- Feuer GA, Shevchuk M, Calanog A; Vulvar Paget's disease: the need to exclude an invasive lesion. Gynecol Oncol. 1990 Jul;38(1):81-9.
- Management of Vulval Cancer, Royal College of Obstetricians and Gynaecologists (2006)
- Creasman WT; Malignant Vulvar Lesions, Medscape, Jun 2013
- FIGO; Staging Vulval cancer. 2001.
- van Doorn HC, Ansink A, Verhaar-Langereis M, et al; Neoadjuvant chemoradiation for advanced primary vulvar cancer. Cochrane Database Syst Rev. 2006 Jul 19;3:CD003752.
- Referral for suspected cancer; NICE Clinical Guideline (2005)
Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.
Dr Hayley Willacy
Dr Hayley Willacy