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. Pruritis vulvae 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.
There are many different causes of vulval lumps and it is important that they are examined properly. Any women with suspicious lumps should be referred for a biopsy.
- Granuloma pyogenicum
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; over the period of a decade the number of diagnoses of infectious syphilis made at genitourinary medicine clinics in England increased by 61%.
- Invasive disease of the vulva.
- Vulval intraepithelial neoplasia (VIN).
- Paget's disease of the vulva.
Other ulcerative conditions
Pre-malignant disease of the vulva
Vulval intraepithelial neoplasia (VIN)
- VIN is considered a pre-malignant state. It can occur by means of cell transformation in already existing vulval disorders such as lichen sclerosus and squamous cell hyperplasia or it can occur independently.
- Screening tests are not available for VIN.
- VIN is a histological diagnosis and a biopsy must be taken.
- There are two types of VIN:
- Usual type:
- Higher incidence in younger women.
- Human papillomavirus (HPV)-related
- Women with usual-type VIN are at a higher risk of developing another HPV-related malignancy of the anogenital tract.
- Current prophylactic HPV vaccines offer protection against usual-type VIN and related invasive carcinoma.
- Differentiated type:
- More common in older patients with chronic dermatological conditions.
- Greater invasive potential and shorter time between diagnosis and squamous cell carcinoma (SCC) than usual-type VIN.
- Not HPV-related.
- Most women with VIN have pruritus, but some are asymptomatic. The lesions may be white, grey, red or raised.
- The diagnosis of VIN is carried out by identifying a lesion by visual inspection and confirming by performing a biopsy.
- Management: biopsy is performed before laser therapy, to make sure that a lesion does not contain invasive cancer.
- High rates of recurrence are associated with smoking, larger lesion size, and positive margins.
- Conventional treatment is wide local excision. Because of the close association of VIN with HPV infection, lifelong follow-up is required to watch for recurrence.
- Imiquimod appears to be effective as an alternative or adjunct to surgery.
- As some lesions spontaneously regress, some women have no active treatment. This may be the best policy for partial-thickness VIN. However, there is a risk of progression and women should be made aware of this.
Malignant disease of the vulva
Paget's disease of the vulva
- Extra-mammary Paget's disease is a rare form of superficial skin cancer. However, the most common site of involvement is the vulva.
- A woman with Paget's disease of the vulva may present with pruritus and weeping or bleeding of the lesion.
- The lesion appears to have an eczematous or velvet-like surface.
- Surgical excision is the gold-standard treatment; however, recurrence rates are high and extensive excisions can produce long-lasting cosmetic and functional defects.
- Alternative treatments include photodynamic therapy, laser therapy, radiotherapy, topical treatments (eg, imiquimod) or chemotherapy.
Vulval cancer is a very rare disease and, on average, a general practitioner will only see a new case once every seven years. There should be a high index of suspicion for abnormal lesions on the vulva, including 'warts' in the postmenopausal woman. See the separate article Vulval Cancer for more information.
- Vulval cancer occurs in about 3.7 per 100,000 women-years in the UK.
- With the exception of the rare sarcomas, this cancer appears most frequently in women aged 65-75 years. However, the incidence in women aged 40-49 years has risen two-fold over the a period of three decades.
- 85% are SCCs, and fewer than 5% are melanoma - the second most common type.
- SCCs can arise on a background of atrophic changes such as lichen sclerosus, or in hypertrophic epithelium.
- 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; however, in advanced-stage primary vulval cancer, treatment is tailored to individual patient needs.
- Radiotherapy, with or without chemotherapy, is increasingly used in the management of advanced vulval cancer.
- The five-year survival in women with no lymph node involvement is more than 80%. This falls to less than 50% if the inguinal nodes are involved and 10-15% if the iliac or other pelvic nodes are involved.
NB: the risk of developing invasive disease in women with lichen sclerosus is approximately 4%. It is not clear whether this risk is reduced by treatment. Women with uncomplicated lichen sclerosus do not require routine hospital-based follow-up, but should be informed of the risks of invasion.
- 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 vulval 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 usually 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 or gynaecology or dermatology, based on appearance and suspicions of the examining GP.
Further reading & references
- Recent epidemiology of infectious syphilis and congenital syphilis; Health Protection Agency, 2013
- The Management of Vulval Skin Disorders; Royal College of Obstetricians and Gynaecologists (February 2011)
- Reyes MC, Cooper K; An update on vulvar intraepithelial neoplasia: terminology and a practical approach to diagnosis. J Clin Pathol. 2014 Apr;67(4):290-4. doi: 10.1136/jclinpath-2013-202117. Epub 2014 Jan 7.
- Preti M, Scurry J, Marchitelli CE, et al; Vulvar intraepithelial neoplasia. Best Pract Res Clin Obstet Gynaecol. 2014 Oct;28(7):1051-62. doi: 10.1016/j.bpobgyn.2014.07.010. Epub 2014 Jul 18.
- Wallbillich JJ, Rhodes HE, Milbourne AM, et al; Vulvar intraepithelial neoplasia (VIN 2/3): comparing clinical outcomes and evaluating risk factors for recurrence. Gynecol Oncol. 2012 Nov;127(2):312-5. doi: 10.1016/j.ygyno.2012.07.118. Epub 2012 Aug 4.
- Kaushik S, Pepas L, Nordin A, et al; Surgical interventions for high-grade vulval intraepithelial neoplasia. Cochrane Database Syst Rev. 2014 Mar 4;3:CD007928. doi: 10.1002/14651858.CD007928.pub3.
- Pepas L, Kaushik S, Bryant A, et al; Medical interventions for high grade vulval intraepithelial neoplasia. Cochrane Database Syst Rev. 2011 Apr 13;(4):CD007924. doi: 10.1002/14651858.CD007924.pub2.
- UK National Guideline on the Management of Vulval Conditions; British Association for Sexual Health and HIV (2014)
- Edey KA, Allan E, Murdoch JB, et al; Interventions for the treatment of Paget's disease of the vulva. Cochrane Database Syst Rev. 2013 Oct 26;10:CD009245. doi: 10.1002/14651858.CD009245.pub2.
- Sanderson P, Innamaa A, Palmer J, et al; Imiquimod therapy for extramammary Paget's disease of the vulva: a viable non-surgical alternative. J Obstet Gynaecol. 2013 Jul;33(5):479-83. doi: 10.3109/01443615.2013.790348.
- Guidelines for the Diagnosis and Management of Vulval Carcinoma; Royal College of Obstetricians and Gynaecologists (May 2014)
- Vulvar Cancer Stages - FIGO System
- 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 Louise Newson
Dr Helen Huins