Differential diagnosis of vulval ulcers

Author: Dr Estella Janz-Robinson, Resident Medical Officer, ACT Health, Canberra, Australia. DermNet New Zealand Editor in Chief: Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy editor: Maria McGivern. April 2017.

What are vulval ulcers?

Vulval ulcers (sores or erosions) are breaks in the skin or mucous membranes of the vulva that expose underlying tissue. They may be itchy or painful. They may produce a discharge. Alternatively, they may be completely asymptomatic.

Who gets vulval ulcers?

Any woman or girl may develop vulval ulcers, irrespective of their age, region, race, ethnicity, sexual preference or socioeconomic status (depending on the cause of the ulcer).

The global incidence of genital ulcer disease is estimated to be more than 20 million cases annually.

What causes vulval ulcers?

Vulval ulcers are the result of tissue death from focal inflammation. They may be caused by both infectious or non-infectious causes.

Infectious causes of vulval ulcers

The infectious causes of vulval ulcers include sexually transmitted infections (STIs) and non-sexually transmitted infections.

Sexually transmitted infections

STIs that can cause vulval ulcers include:

Non-sexually transmitted infections

Viral infections that can cause vulval ulcers include:

Bacterial infections that can cause vulval ulcers include:

The most common fungal infection that can cause vulval ulcers is vulvovaginal candidiasis.

Non-infectious causes of vulval ulcers

Non-infectious causes of ulceration of the vulva include aphthosis, inflammatory diseases, blistering diseases, and malignancies.

Vulval aphthosis

After herpes simplex, aphthosis or non-sexually acquired genital ulceration is the second most common cause of vulval ulcers, with the highest rates occurring in Caucasians and adolescents.

Other names for vulval aphthosis include vulval aphthous ulcers, Lipschütz ulcer, Mikulicz ulcer, Sutton ulcer, and ulcus vulvae acutum. Vulval aphthosis is commonly associated with oral ulceration.

Aphthous vulval ulcers may be reactive — following an infection (such as infectious mononucleosis) or trauma — or be related to an underlying systemic disease such as:

Inflammatory diseases

A range of autoimmune and autoinflammatory diseases may present with vulval ulceration. These include:

Blistering diseases

Autoimmune blistering diseases may present with erosions and ulcers. The vulva is rarely the only site affected. 

Genetic diseases can present with chronic ulceration of vulval and perianal skin.

Malignancy

Malignancies that can cause ulceration of the vulva include:

Less commonly, the following can also cause ulceration of the vulva:

What are the clinical features of vulval ulcers? 

Vulval ulcers are often grouped by the following features:

Typical characteristics of various presentations with vulval ulceration are described below.

Herpes simplex virus

Chancroid

Primary chancre of syphilis

Lymphogranuloma venereum

Granuloma inguinale

Aphthosis

Crohn disease

Pyoderma gangrenosum

Hidradenitis suppurativa

Squamous cell carcinoma

How are vulval ulcers diagnosed?

Diagnosis of vulval ulceration involves taking a careful history and performing a physical examination to assess the risk of STIs, guide appropriate investigations, and determine the need for empirical therapy.

It is important to consider that:

No pathogen is identified in up to 25% of patients; however, the aim of initial investigations is usually focused on diagnosing STIs. Patients should, as a minimum, have the following investigations:

Since co-infections are common and many STIs are asymptomatic, patients with recent unprotected sexual contact should also be tested for non-ulcerative STIs via:

The geographic location of the STI acquisition, the individual’s sexual and travel history, and the local prevalence of chancroid, LGV and granuloma inguinale should be considered prior testing for these STIs.

In patients with a low risk of STIs or in those who have had negative results, depending on the clinical presentation, it is reasonable to consider:

Biopsy may be necessary if:

What is the treatment for vulval ulceration?

General measures

Whatever the cause, patients with vulval ulcers may need education, reassurance and symptom relief.

Affected individuals can:

Oral antihistamines may also be beneficial in certain cases.

Note: severe pain and urinary retention may require hospitalisation and catheterisation.

Specific treatment

Treatment of infectious causes may include:

Note: empirical treatment is initiated when there has been a known exposure to an STI, genital ulcers are suggestive of HSV, there is a high risk for syphilis, or when failure follow-up for treatment is likely.

Treatment of non-infectious causes may include:

Note: many conditions involving vulval ulceration require a multidisciplinary approach.

What is the outcome for vulval ulceration?

The prognosis of vulval ulcers depends on the cause.

If left untreated, vulval ulcers can have serious health implications, including:

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