Pemphigus vulgaris is a rare autoimmune disease that is characterised by blisters and erosions on the skin and mucous membranes, most commonly inside the mouth. It is the most common subtype of pemphigus, accounting for 70% of all pemphigus cases worldwide although it is extremely rare in New Zealand (about one case per million). The other two main subtypes of pemphigus are pemphigus foliaceus and paraneoplastic pemphigus.
Pemphigus vulgaris is an autoimmune blistering disease, which basically means that an individual's immune system starts reacting against his or her own tissue.
The building block cells of the epidermis are called keratinocytes. These cells are cemented together at special sticky spots called desmosomes. In pemphigus vulgaris immunoglobulin type G (IgG) autoantibodies bind to a protein called desmoglein 3, which is found in desmosomes in the keratinocytes near the bottom of the epidermis. The result is the keratinocytes separate from each other, and are replaced by fluid, the blister.
Pemphigus vulgaris affects people of all races, age and sex. It appears most commonly between the ages of 50-60 years, and is more common in Jews and Indians presumably for genetic reasons.
Most patients first present with lesions on the mucous membranes such as the mouth and genitals. Several months' later blisters on the skin may develop or in some cases mucosal lesions are the only manifestation of the disease.
The most common mucosal area affected is the inside of the mouth but others include the conjunctiva, oesophagus, labia, vagina, cervix, penis, urethra and anus. Common features of oral mucosal pemphigus include:
- 50-70% of patients get oral lesions
- blistering superficial and often appears as erosions
- widespread involvement in the mouth
- painful and slow to heal
- may spread to the larynx causing hoarseness when talking
- may make it difficult to eat or drink
Skin lesions appear as thin walled flaccid blisters filled with clear fluid that easily rupture causing painful erosions. Erosions in the skin folds may develop into vegetative lesions which are granular and crusty looking (known as pemphigus vegetans).
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