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Facts about the skin from DermNet New Zealand Trust. Topic index: A B C D E F G H I J K L M N O P Q R S T U V W X Y Z



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?

Site Features
Eye
  • Sensation of grittiness or pain
  • Conjunctivitis
  • Lesions form, erode and heal to leave scar tissue
  • May lead to impaired vision or blindness
Mouth
  • Blisters form first on the gums near the teeth
  • Palate, tongue, lips, buccal mucosa, floor of the mouth and throat may be affected
  • Painful erosions and ulcers make it difficult to eat
  • Weight loss and nutritional deficiencies may develop due to starvation
  • Lesions occurring in the throat (oesophagus, trachea and larynx) can become life-threatening
Skin
  • Blisters on the skin develop in 25–30% of patients
  • May be itchy
  • Bleeding may occur if traumatised
Nose
  • Nose bleeds after blowing the nose
  • Crusting causing discomfort
Genitals
  • Painful blisters, erosions and swelling of the clitoris, labia, and anus in women, and of the penis and anus in males
  • Scarring can lead to severe distortion of genitalia
  • May cause difficulty and pain passing urine
  • Adhesions can lead to narrowing or even closure of the vaginal orifice
Cicatricial pemphigoid Cicatricial pemphigoid Cicatricial pemphigoid
Cicatricial pemphigoid Cicatricial pemphigoid Cicatricial pemphigoid
Mucous membrane pemphigoid

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.

Organ Specific treatments
Mouth, nose and skin
Eye
  • Corticosteroid eye drops
  • Surgery to release adhesions
Oesophagus, trachea and larynx
  • Soft or pureed diet
  • Topical steroids
  • Dilation of strictures
Vulva, vagina and anus
  • Greasy emollient such as petroleum jelly applied frequently
  • Local ultrapotent topical corticosteroid (eg clobetasol ointment)
  • Oestrogen cream intravaginally
  • Use vaginal dilators to prevent adhesions
  • Surgery to divide adhesions if severe
  • Surgery must be followed by intense use of topical corticosteroids and dilators to prevent recurrence of adhesions

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:

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.

Related information

References:

  1. Definitions and outcome measures for mucous membrane pemphigoid: recommendations of an international panel of experts. J Am Acad Dermatol. 2015 Jan;72(1):168-74. doi: 10.1016/j.jaad.2014.08.024. Epub 2014 Nov 4. Open Access article

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Author: Vanessa Ngan, staff writer (2003). Updated by Hon Assoc Prof Amanda Oakley, March 2015.


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