Background

Vulvar ulcers are rare in girls and young women, especially when they are not sexually active.Most lesions are exquisitely painful and result in considerable anxiety and emotional distress for both the patient and family, not to mention the physician's frustration in trying to expediently diagnose and treat a lesion which is rarely seen in general practice.Parents and physicians may also suspect sexual abuse, which can be very disconcerting.  Most ulcers in the paediatric population however are NOT sexually transmitted infections. 

The differential diagnosis of non-sexually transmitted vulvar ulcers is as follows (most common in bold)

• Aphthous ulcers(synonyms include aphthosis, canker sores, Lipschutz ulcers, ulcus vulvae acutum)
• Infectious
  • HSV via autoinoculation
  • EBV (self limited genital ulcers)
  • CMV
  • VZV (varicella or herpes zoster)
  • Group A Strep
  • Mycoplasma
  • Molluscum contagiosum
• Autoimmune
  • Crohn's disease (ulcers + GI symptoms)
  • Behçet's disease (Aphtous genital ulcers that last for weeks and heal with scarring)
  • Vaculitis (LUPUS)
  • Pemphigus and Pemphigoid (lesions may mimic lichen sclerosus with extensive scarring)
• Drug reactions
  • Fixed drug eruptions(NAIDs, metronidazole, Acetaminophen, Sulfonamides, Tetracycline, Phenitoyn, oral contraceptives, Barbiturates, Phenolphthaten)
  • Stevens Johnson's syndrome/ toxic epidermal necrolysis
• Other
  • Erosive Lichen Sclerosus
  • Hair removal folliculitis
  • Epidermolysis bullosa
  • Allergic contact dermatitis 

In sexually active young women, or in cases of sexual abuse, the differential diagnosis also includes:

• HSV (most common, multiple vesicles progressing to pustules over 10-14 days)
• Syphilis (painless ulcer)
• Rarely- Lymphogranuloma venereum and Chancroid ( Ulcer is exquisitely painful and is associated with suppurative inguinal adenopathy) 

Assessment

History:

The history should determine whether these are primary or recurrent lesions and the evolution of it.  A review of systems should include:

Systemic symptoms (fever, malaise, headache, GI symptoms, respiratory symptoms, myalgia)

Oral ulcers, skin lesions, ocular symptoms.

A family history of autoimmune disorders should be determined.  Behçet's disease is more common in families from Mediterranean countries, Turkey and the Middle East, and South East Asia.

Social history should be obtained confidentially, especially if there is a possibility of sexual activity. 

Examination:

Assess for the following:

• General appearance
• Oral ulcers
• Uveitis (Behçet's)
• Skin inspection (eczema, rash, other ulcers or bullae)
• Lymphadenopathy (cervical, inguinal/femoral)
• Size, shape, location of vulvar ulcer(s): Physical examination of the external genitalia can be accomplished in the frog leg or knee chest position. 

Investigations:

(send results to GP)

Non-sexually active:

• Swabs:
  • HSV 1 and 2, VZV
    • PCR
    • OR immunofluorescence
    • OR culture
  • Gram stain and bacterial culture
  • Yeast Culture 

• Consider the following
  • Serology:
    • Herpes simplex (HSV-1/HSV-2)
    • EBV (IgM and IgG) and/ or monotest
    • CMV and Mycoplasma
  • CRP and ESR
  • FBE
  • ANA and Consider HLA-B51 if Behçet's disease is a possibility 

Sexually active:

• All of the above plus:
• Urine:
  • Pregnancy test
  • PCR for Chlamydia (First pass urine test)
  • PCR for Gonorrhea (First pass urine test)
• Serology:
  • RPR (Rapid Plasma Reagin)
  • HIV 

If non healing refer the patient for biopsy of lesion.

Management

Because of the delay in results from most of the investigations, treatment is often supportive, directed to pain relief, prevention of scarring and specific treatment based on diagnosis.  In severe cases where micturition is impossible secondary to pain, an in-dwelling catheter may be required. 

Provide reassurance to parents and the patient that ulcers in young girls and women are often not sexually transmitted, and that recurrence rates are low. 

1. Remove all irritants
2. No pads, underwear, tight clothes
3. Pain relief: 
   1. Salt baths and general vulvar hygiene are recommended (see vulvar care handout).
   2. Spray bottles or voiding in the bath to reduce external dysuria
   3. Petrolatum or zinc oxide barrier after soak
   4. Topical anaesthetics (ie. Viscous Xylocaine, Lignocaine gel 2%)
   5. Oral Analgesics ( NSAIDs)
4. Antiviral:
   1. Consider Aciclovir if primary HSV likely diagnosis
5. Steroids:
   1. Consider topical corticosteroids (Advantan fatty ointment) if aphthous ulcers or Behçhet's disease most likely
6. Antibiotics: if bacterial infection suspected
7. Treatment of Sexually Transmited Infections (Refer to CPGs on STIs)
8. Immunosuppressants: (Discuss with Reumathology Consultant if Behchet's disease and aphtous ulcers is likely the diagnosis) 

Referral

Most ulcers are self-limiting and can be followed up by the GP.  If the ulcers are recurrent or there are associated abnormalities to the underlying skin, then a referral to Gynaecology for follow-up should be organized.  If Behçet's disease is suspected, a referral to Rheumatology may be useful.  Dermatology or Infectious Diseases could also be consulted.