Bullous disorders of the mouth

What is it about ?

In addition to other physical symptoms, a number of conditions cause blisters to form in the mouth. These conditions belong to the group of autoimmune chronic bullous diseases.

An autoimmune disease is a disease of the immune system in which the body makes antibodies against its own proteins. Normally, antibodies are only produced to defend the body against outside agents, such as bacteria or cancer cells. In the case of an autoimmune disease, an error occurs in the production of these antibodies which, as a result, consider the organism as a foreign body and attack it as such.

The adjective bullous means that there is formation of vesicles. In these conditions, the body produces antibodies against proteins in the mucous membranes of the mouth, which causes blisters to form. These are chronic conditions, which can recur.

The two main bullous conditions of the mouth are pemphigus (vulgar pemphigus) and the pemphigoid, the latter further subdividing into bullous pemphigoid and benign mucous membrane pemphigoid.

What is their frequency?

Pemphigus and pemphigoid are very rare conditions, mostly seen in older people.

the pemphigus mainly occurs between the ages of 40 and 60 and affects women somewhat more often than men. Each year, between 0.1 and 1.6 people in 100,000 are affected by pemphigus (1).

The pemphigoid most commonly affects people aged 60-70, and it is as common in men as it is in women. Every year, 0.6 to 0.7 people in 100,000 develop a bullous pemphigoid. The frequency is even lower for the benign mucous membrane pemphigoid, with 0.1 in 100,000 (2, 3).

How to recognize them?

Pemphigus (vulgar pemphigus)

Pemphigus is bullous in more than 50% of cases. The blisters that form in the mouth hardly ever stay intact because they burst easily and leave small, painful sores. The blisters are mainly visible on the cheek, palate, floor of the mouth, or lips.

Pemphigoid

The vesicles formed in the mouth are very fragile and filled with fluid. The mucous membrane around them is red. When they burst, they leave a small painful sore, which slowly heals, forming a scar. In the case of bullous pemphigoid, the risk of developing these oral lesions is 10 to 20%. In the case of a benign pemphigoid of the mucous membranes, this risk is 85%. In addition to this blistering, the gums are also usually red, inflamed, and ulcerated. Other mucous membranes in the body, such as the eyes or bladder, can also be damaged. If your doctor notices eye problems, they will refer you to an eye specialist (ophthalmologist).

How is the diagnosis made?

Your doctor will review your history and current treatments, and then ask you questions about your symptoms. If he suspects any of these conditions, he will refer you to a skin specialist (dermatologist). This specialist will carefully examine the lesions on the skin and in the mouth and possibly perform the Nikolsky test. To do this, the dermatologist presses a flat object against the healthy lining that surrounds a gallbladder for one minute. If a new blister forms, this is a characteristic sign of pemphigus. The doctor can thus exclude an infectious origin. The diagnosis can then be confirmed with a blood test and, if necessary, a biopsy. This examination involves taking a small piece of tissue from the lesion and having it examined in the laboratory.

What can you do ?

Try to avoid any irritation to the skin or mucous membranes, which could cause blisters to form.

• Avoid hard foods.
• Protect yourself against sunburn.
• Avoid wounds or skin lesions due to burns, abrasions, …
• Dry yourself thoroughly after showering.
• Avoid certain medicines, eg a number of medicines for psychosis and water pills.
• Do not pierce the blisters.
• Treat wounds left by burst blisters properly to prevent infection.

What can your doctor do?

Treatment depends on the severity of the condition.

Pemphigus

In the face of pemphigus, corticosteroids and / or immunosuppressants are started more quickly in order to deactivate the antibodies that attack the cells of the body. Since this is a chronic condition, treatment should be taken for a long time to avoid recurrence.

Pemphigoid

Oral lesions can be treated with a gel, ointment or cream. If the effect is not sufficient or if the condition is severe, a course of corticosteroids (tablets) will be prescribed. Oral administration causes more side effects than an ointment. This treatment is usually sufficient for pemphigoid, and medications can often be stopped gradually.

Want to know more?

• Pemphigus (image) – DermIS – Dermatology Information System
• Bullous pemphigoid (image) – DermIS – Dermatology Information System
• Benign mucous membrane pemphigoid (image) – DermIS – Dermatology Information System

• Sun protection – Christian mutuality

• Corticosteroids – CBIP – Belgian Center for Pharmacotherapeutic Information

Sources

• Foreign clinical practice guide ‘Orale aandoeningen met blaarvorming’ (2017), updated on 22.03.2017 and adapted to the Belgian context on 21.01.2019 – ebpracticenet
• International Pemphigus Pemphigoid Foundation (IPPF) | a common hope an uncommon bond [Internet]. [cited 2019 Mar 28]. Available on : http://www.pemphigus.org/
• (1) Martin LK, Agero AL, Werth V, Villanueva E, Segall J, Murrell DF. Interventions for pemfigus and pemphigus foliaceus. Cochrane Database Syst Rev. 2009 Jan 21; (1).
• (2) Kirtschig G, Middleton P, Bennett C, Murrell DF, Wojnarowska F, Khumalo NP. Interventions for bullous pemphigoid. Cochrane Database Syst Rev. 2010 Oct 6; (10).
• (3) Kirtschig G, Murrell DF, Wojnarowska F, Khumalo NP. Interventions for mucous membrane pemphigoid and epidermolysis bullosa acquisita. Cochrane Database Syst Rev. 2003 Jan 20; (1).