Differential diagnosis of vulvar ulcers

What are vulvars ulcers?

Vulvar ulcers (sores or erosions) are breaks in the skin or mucous membranes of the vulva that expose the underlying tissue. They can cause itching or pain. They can produce a download. Alternatively, they can be completely asymptomatic.

Who gets vulvar ulcers?

Any woman or girl can develop vulvar ulcers, regardless of 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 at more than 20 million cases a year.

What causes vulvar ulcers?

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

Infectious causes of vulvar ulcers

Infectious causes of vulvar ulcers include sexually transmitted infections (STIs) and non-sexually transmitted infections.

Sexually transmitted infections

STIs that can cause vulvar ulcers can include:

• Genital herpes: caused by the herpes simplex virus (HSV); HSV 2 is more common than HSV I

• Primary syphilis - caused by Treponema pallidum

• Chancroid - caused by Haemophilus ducreyi

• Lymphogranuloma venereum (LGV) - caused by Chlamydia trachomatis L1 and L2 serotypes

• Granuloma inguinal (donovanosis) - caused by Klebsiella granulomatis.

Non-sexually transmitted infections

Viral infections that can cause vulvar ulcers include:

• Herpes simplex via autoinoculation

• Epstein-Barr virus (EBV)

• Cytomegalovirus (CMV)

• Chicken pox or Herpes zoster (shingles) – caused by the varicella-zoster virus (VZV).

Bacterial Infections that can cause vulvar ulcers include:

• Group A streptococcal infections

• Mycoplasma

The most common fungus. infection which can cause vulvar ulcers is vulvovaginal yeast infection.

Non-infectious causes of vulvar ulcers

Non-infectious causes of ulceration of the vulva include aphtosis, inflammatory malignant diseases, blisters and tumors.

Vulvar aphtosis

After herpes simplex, aphtosis or sexually not acquired genital ulceration is the second most common cause of vulvar ulcers, with the highest rates occurring in Caucasians and adolescents.

Other names for vulvar aphtosis include vulvar aphthous ulcers, Lipschütz ulcer, Mikulicz ulcer, Sutton ulcer, and ulcus vulvae acutum. Vulvar aphtosis is commonly associated with oral ulceration.

Aphthous vulvar ulcers may be reactive after infection (such as infectious mononucleosis) or trauma - or be related to an underlying systemic disease like:

• Crohn's disease
• Behçet's disease

• Gluten enteropathy (celiac disease)

• Systemic lupus erythematosus

• Human immunodeficiency virus (HIV) infection

• Myeloproliferative disorder.

Inflammatory diseases

A range of autoimmune disease and autoinflammatory Diseases can present with vulvar ulceration. These include:

• Dermatitis - due to scratching or secondary infection
• Erosive lichen planus
• Lichen sclerosus

• Fixed drug eruption - most commonly caused by non-steroids anti-inflammatory drugs (NSAID), paracetamol, sulfonamides and tetracyclines

• Stevens - Johnson syndrome (SJS) / toxic epidermal necrolysis (TEN)

• Crohn's disease: usually presents with linear ulcers

• Systemic lupus erythematosus

• Darier's disease
• Pyoderma gangrenous

• Hidradenitis suppurativa.

Blistering diseases

Autoimmune blistering skin conditions can present with erosions and ulcers. The vulva is rarely the only affected site.

• Pemphigus vulgaris is an erosive disease that leaves no scars. Oral mucous membrane, vulva, anus, and scalp are common sites of involvement.

• Mucous membrane pemphigoidscar pemphigoid) causes chronic ulceration and scarring. It usually affects more than one. mucous membrane site that includes the vulva, anus, mouth, eyes, nostrils and can also affect the scalp.
• Bullous pemphigoid produces time bullas (fluid-filled blisters) on normal skin or, more often, eczematous or Urticated plates. It rarely involves mucous surfaces, but is common in body folds (i.e., the back of the knees, the inside of the armpits, elbows, and groin) in older people.
• Erythema multiform is a acute or recurrent reactive condition Erythema multiforme major can cause very painful vulvar ulceration, generally associated with oral ulceration and target lesions in the distal extremities

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

• Epidermolysis bullosa is a group of disorders that present with blisters in childhood. Subtypes of epidermolysis bullosa that can cause vulvar ulcers include junctional epidermolysis bullosa and dystrophic epidermolysis bullosa.

• Family benign Chronic pemphigus (Hailey-Hailey disease) occurs in early adulthood with chronic disease, symmetrical maceration and erosions of the vulva, perianal skin, and others push-ups, even under the breasts and neck.

Malignancy

Malignant tumors that can cause ulceration of the vulva include:

• Scaly cell carcinoma

• Intraepithelial vulval neoplasm (vulvar squamous intraepithelial lesions).

Less often, the following may also cause ulceration of the vulva:

• Extramammary Paget's disease
• Basal cell carcinoma
• Cell B lymphoma
• Leukemia skin

• Langerhans cell histiocytosis (histiocytosis X).

What are the clinical characteristics of vulvar ulcers?

Vulvar ulcers are often grouped for the following characteristics:

• Single or multiple
• Painful or painless
• Ulcer evolution (i.e. initial appearance and progression)
• Ulceration preceded by dryness, climbingand excoriations suggests dermatitis
• Frequency of episodes
• Recurrent ulcers may suggest HSV, Behçet's disease, fixed drug eruption
• Associated signs or symptoms
• Inguinal lymphadenopathy indicates probable infection
• Uveitis, arthritis and / or a family history of these may suggest Behçet's disease
• Exposure to new medications may indicate a fixed drug eruption
• Oral mucosa involvement occurs in aphtosis or erosive lichen planus
• Dysuria may be due to the location of the ulcer or sexual transmission urethritis
• Constitutional symptoms can occur in herpes simplex, secondary syphilis, LGV, or systemic lupus erythematosus.

The typical characteristics of various presentations with vulvar ulceration are described below.

Herpes simplex virus

• Clusters of small blisters. get together and it opens to form painful (or itchy) sores with a red base.
• Ulcers may appear on the vulva, cervix, vagina, perineum, legs or buttocks.
• AtypicalExtensive or long-lasting disease affects immunosuppressed patients.
• Big and tender jock lymph nodes and 'flu-like symptoms occur with a primary infection.

Chancroid

• Single or multiple, unilateralpainful papules become pustular and ulcerate
• The ulcers show irregular torn edges with gray or yellow discharge.
• As the disease progresses, approximately 50% of patients develop sensitive inguinal lymph nodes that can rupture and cry.

Syphilis primary chancre

• There is a single painless ulcer with a clean base and firm raised edges.
• It is often associated with large, non-sensitive inguinal lymph nodes.

Venereal lymphogranuloma

• There is a single, transient and painless ulcer.
• It is often associated with urethritis and is followed by sensitive inguinal lymph nodes weeks later.

Inguinal granuloma

• There are single or multiple, chronic, red, induratedPainless, ulcers that bleed easily.

Aphthosis

• There are intensely painful stabbing ulcers, which are often bilateral, with a yellow-white base and red edges.
• There is associated lip swelling, severe pain and dysuria, and sometimes large, tender inguinal lymph nodes.
• Recurrent episodes of ulceration of the genital and oral mucosa associated with uveitis suggest Behçet's disease.

Crohn's disease

• Mixed inflammatory lesions, fissures, and there are "knife cut" ulcers of varying severity.
• Deeper ulcers can progress to fistulas; more commonly, in perianal or rectovaginal sites.
• Painless vulvar marking edema it can happen.

Gangrenous pyoderma

• A tender inflammatory pustule evolves rapidly in a deep purulent ulcer with violaceous undercut edges and possible satellite injury.
• Often precipitate from a minor injury at the ulceration site.

Hidradenitis suppurativa

• It comes with pseudocystsinflammatory nodules, sinus drainage and abscesses.
• Ulceration may be associated with pyogenic granuloma-like nodules.
• The skin on the labia majora, pubis, thighs, buttocks, and under the armpits and breasts may be affected.

Squamous cell carcinoma

• A magnification, irregular red, pink, or white nodule or license plate It has a wart-like and / or ulcerated surface.
• Squamous cell carcinoma can arise within intraepithelial neoplasia, lichen sclerosus, erosive lichen planus, or normal skin.

How are vulvar ulcers diagnosed?

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

It's important to put attention on:

• More than one cause can coexist
• There are various presentations of the disease, so the clinical appearance alone can be misleading.
• People with immunosuppression can have atypical presentations.

Not pathogen it is identified in up to 25% of the patients; However, the focus of initial research generally focuses on diagnosing STIs. Patients must, at a minimum, have the following investigations:

• Genital Viral Swabs injury for HSV Polymerase chain reaction (PCR)
• Syphilis blood serology.

Since co-infections are common and many STIs are asymptomatic, patients with recent unprotected sexual contact should also undergo testing for non-ulcer STIs through:

• Urine PCR for chlamydia and gonorrhea
• Bloods for HIV, and hepatitis Serology B and C.

The geographical location of the STI acquisition, the individual's and local's travel and sexual history predominance Chancroid, LGV, and inguinal granuloma should be considered pretests for these STIs.

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

• Bacterial swabs for gram staining and bacterial and yeast culture

• Viral swabs for VZV PCR

• Blood for:

  • HSV (HSV 1 and HSV 2), EBV (immunoglobulins M and G) or a Monospot, CMV and mycoplasma serology test

  • A complete blood count C-reactive proteinand antinuclear antibody tests
  • Test for HLA-B51 if Behçet's disease is a possibility.

A biopsy It may be necessary if:

• A diagnosis cannot be made byinvader methods
• Ulcers do not resolve after standard therapy.
• The injuries are suspected of an underlying disorder or malignancy.

What is the treatment for vulvar ulceration?

General measures

Whatever the cause, patients with vulvar ulcers may need education, peace of mind, and symptom relief.

Affected people can:

• Minimize irritants (no soaps, showers, pads, tight underwear or clothing)
• Consider using spray feeding bottles or evacuating in a bathroom to reduce pain when urinating
• Try salt baths
• Apply cold compresses (eg, a flannel soaked in cold water)
• Use soft barrier ointments (eg, Vaseline, zinc oxide)
• Apply one current anesthetic (eg lignocaine gel, lignocaine / prilocaine cream)
• Use oral pain relievers (acetaminophen, NSAIDs) as needed.

Oral antihistamines can also be beneficial in certain cases.

Note: severe pain and urinary retention may require hospitalization and catheterization.

Specific treatment

Treatment of infectious causes may include:

• Oral acyclovir, famciclovir, or valaciclovir for herpes simplex or zoster
• Intramuscular penicillin G (benzylpenicillin) for primary syphilis
• Intramuscular ceftriaxone or oral azithromycin, ciprofloxacin, or erythromycin for chancroid
• Oral doxycycline for LGV and inguinal granuloma.

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

Treatment of non-infectious causes may include:

• Topical corticosteroids or intralesional corticosteroids.

• Oral corticosteroids

• Antibiotics or antifungal agents in cases of secondary infection.

• Immunomodulator agents such as methotrexate, topical or systemic cyclosporine
• Referral to appropriate specialists (eg, Rheumatologist, dermatologist, gynecologist, sexual health doctor, infectious disease doctor).

Note: Many conditions involving vulvar ulceration require a multidisciplinary approach.

What is the result of vulvar ulceration?

the forecast of vulvar ulcers depends on the cause.

• Most STIs can be cured quickly with the right treatment.

• HSV cannot be cured, but recurrences can be controlled with early recognition and antiviral prophylaxis. The severity and frequency of episodes may decrease over time.

• Non-sexually acquired genital ulcers usually resolve on their own without scarring in 2 to 6 weeks.

Left untreated, vulvar ulcers can have serious health consequences, including:

• Increased risk of HIV transmission for sexually active people
• Persistent latent untreated syphilis infection, resulting in occult transmission to sexual partners and progression to secondary or tertiary syphilis
• Risk of transmission of STIs to a fetus in pregnancy or to a newborn during birth
• Scarring, adhesions or destruction of the vulvar architecture in erosive lichen planus, lichen sclerosus, Behçet's disease, mucous membrane pemphigoid (cicatricial pemphigoid), pemphigus vulgaris, erythema multiforme major, epidermolysis bullosa, and suppurative hydradenitis
• Increased risk of malignancy in areas affected by severe inflammation, such as erosive lichen planus, lichen sclerosus, and occasionally chronic suppurative hydradenitis
• On going psychosocial anguish.