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Cicatrical Pemphigoid - A rare chronic autoimmune subepithelial blistering disease
characterised by erosive skin lesions of the mucous membranes and skin that results
in scarring of at least some sites of involvement .
The autoimmune reaction most commonly affects:
The mouth, causing lesions in the gingiva or gums
It can also affect areas of mucous membrane elsewhere in the body, such as:
The sinuses.
The genitals.
The anus.
The cornea of the eye - repeated scarring may result in blindness.
This chronic, progressive disease responds poorly to treatment. Spontaneous remissions
are rare. Some people may experience long remissions with intermittent exacerbations.
Symptoms:
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 and make it difficult to eat
Lesions occurring in the throat (oesophagus, trachea and larynx) can become life-threatening
Skin
Blisters on the skin develop in 25-30% of people
May be itchy
Bleeding may occur if traumatised
Nose
Nose bleeds after blowing the nose
Crusting causing discomfort
Genitals
Painful blisters and erosions on the clitoris, labia, shaft of the penis, perianal
area.
Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.).
McGraw-Hill
