Mucous membrane pemphigoid

What is mucous membrane pemphigoid?

Mucous membrane pemphigoid is an autoimmune disease that is characterised by blistering lesions on mucous membranes. Areas commonly involved are the oral mucosa (lining of the mouth) and conjunctiva (mucous membrane that coats the inner surface of the eyelids and the outer surface of the eye). Other areas that may be affected include the nostrils, oesophagus, trachea and genitals. Sometimes the skin may also be involved where blistering lesions can be found on the face, neck and scalp.

Mucous membrane pemphigoid is also called cicatricial pemphigoid or oral pemphigoid.

Brunsting Perry cicatricial pemphigoid is a rare variant in which localised crops of recurrent blisters arise within urticarial plaques, usually on the head and neck. The blisters may burst resulting in blood-crusted plaques and scars.

Who gets mucous membrane pemphigoid?

Mucous membrane pemphigoid is predominantly a disease of the elderly with a peak incidence at around 70 years. However, childhood cases have been reported. It appears to be twice as common in women than men.

What are the signs and symptoms of mucous membrane pemphigoid?

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What causes mucous membrane pemphigoid?

Mucous membrane pemphigoid is an autoimmune blistering disease, which basically means that an individual's immune systems starts reacting against his or her own tissue. In this particular instance autoantibodies react with proteins found in mucous membranes and skin tissue resulting in blistering lesions. The binding site appears to be within the anchoring filaments that help the epidermis (outside layer of skin) stick to the dermis (inner layer of skin).

How is mucous membrane pemphigoid diagnosed?

Mucous membrane pemphigoid is suspected from the clinical appearance of blisters and scarring. Diagnosis should be confirmed by biopsy for histopathological examination, and often multiple biopsies are necessary from the edge of blistered or ulcerated tissue.

An additional specimen from adjacent tissue may be examined by direct immunofluorescence to detect immunoglobulins along the basement membrane. Blood samples may be tested for skin antibodies by indirect immunofluorescence.

Patients with severe disease should undergo evaluation of general health and nutrition, as ulceration of mouth and oesophagus can make it difficult to eat.

The severity of the mucous membrane pemphigoid can be assessed using the mucous membrane pemphigoid disease area index (MMPDAI).¹

Treatment of mucous membrane pemphigoid

The primary aim of treatment is to stop blister formation, promote healing and prevent scarring. Mucous membrane pemphigoid is a particularly difficult disease to treat as it can affect so many different parts of the body. Usually a team of medical specialists are required for overall care. Regular examinations are necessary.

Local medical treatment is very much dictated by the organs involved.

Systemic therapy for mild disease may include a tetracycline antibiotic (doxycycline, minocycline, lymecycline), or less often, colchicine, nicotinamide, salazopyrin, sulfapyridine or sulfamethoxypyridazine.

Systemic therapy for severe disease usually involves a combination of systemic corticosteroids (sometimes in high dose), dapsone and immunosuppressive agents, particularly:

• Cyclophosphamide
• Azathioprine
• Mycophenolate
• Methotrexate
• Ciclosporin
• Rituximab.

Usually a combination of medications are used and treatment is slowly effective. The eventual aim is to reduce the dose of systemic steroid while maintaining immunosuppression. Long term treatment and monitoring is usually required.

Appropriate wound care is particularly important to promote healing and minimise scarring.

Mucous membrane pemphigoid is a chronic, progressive disease that responds slowly and often incompletely to treatment. Spontaneous remissions are rare.