Vesiculobullous conditions

What are vesiculobullous conditions?

Vesiculobullous conditions derive their name from the manifestations of “bubbles” forming in the oral mucosa (and/or skin and other mucosal surfaces e.g. conjunctivae) which may be small (vesicles) or large (bullae). The term is something of an umbrella for a number of conditions including (but not limited to):

• pemphigus vulgaris: characterised by intra-epithelial bullae that easily rupture, this may affect skin, as well as other mucosal surfaces e.g. oesophageal, genital, conjunctivae
• paraneoplastic pemphigus: a rare disease of sudden onset that is associated with neoplastic conditions (e.g. non-Hodgkin’s lymphoma)
• cicatricial pemphigoid: also known as mucous membrane pemphigoid (MMP) this can affect cutaneous and mucosal surfaces with subepithelial bullae that are more robust – an early diagnosis reduces complications
• linear IgA disease: a rare autoimmune condition with heterogenous eruptions resembling pemphigoid but attributable to IgA autoantibodies, this may be triggered by certain medications
• acquired bullous epidermolysis (ABE): a rare chronic subcutaneous dermatosis with an autoimmune aetiology suggested by the presence of IgG autoantibodies to type VII collagen, if left unchecked resultant scarring can cause significant complications

How are they diagnosed?

Diagnosis begins with clinical suspicion based on years of experience and pattern recognition. I may choose to involve other specialties early on, particular when other sites are affected, particularly dermatologists when skin manifestations are also present. Ultimately, a biopsy is usually required. This can easily be performed in the outpatient setting and consists of removing a representational portion of tissue (in some instances peri-lesional or unaffected tissue) under local anaesthesia (awake). The area is repaired with resorbable (dissolving) sutures, and specimens will be sent for special immunofluorescence testing to look for key autoantibodies. This means an accurate diagnosis and early treatment, translating into the best outcomes for my patients.

What treatment options are there?

The mainstay of management for many of these conditions is steroid therapy. In milder forms, we may be able to treat the condition with topical low potency steroids, moving to high potency steroids if these are ineffective. In more aggressive or advanced cases, systemic steroids can be prescribed although these can have side effects that require careful titration of doses and monitoring, often working hand in hand with your General Practitioner (GP) to look after you. A minority of patients will require steroid-sparing or immunosuppressive therapies (e.g. tacrolimus, azathioprine). These dampen the immune response and reduce inflammation and autoimmune diseases, but again have a number of side effects that require regular appointments and careful monitoring.

More broadly, it is important to consider:

• good oral hygiene;
• working in conjunction with your general dental practitioner to optimise periodontal health and restorations;
• avoiding smoking and alcohol consumption;
• treatment of any superinfection e.g. antifungals (where required);
• stress reduction;
• medication reviews.

Do any other specialties need to be involved?

If required, I will involve other specialties early in your treatment. This may be as a result of the complexity of your treatment, co-existing medical conditions, the frequency of treatment monitoring required and/or the areas of the body that are affected. These clinicians may include specialists in the field of:

• dermatology;
• oral medicine;
• gynaecology;
• ophthalmology.