Pemphigus refers to a group of skin disorders that cause blistering and sores on the skin or mucous membranes of the mouth, nose, throat or genital region.
The onset of disease is usually in mid-to-late adulthood, although the condition may also develop in younger adults and children.
As a chronic condition, pemphigus is most easily controlled when it is diagnosed and treated early in the disease course.
The treatments used may be similar to the care given for severe burns.
The most common type of pemphigus is pemphigus vulgaris. Pemphigus vegetans is a rare and benign variant of pemphigus vulgaris that has a much better prognosis.
Pemphigus vulgaris
Pemphigus vulgaris is an autoimmune disease. The immune system mistakes the skin and mucous membrane cells in the body as foreign and launches an attack against them, as if it were fighting an infection.
These antibodies are referred to as autoantibodies. Autoantibodies bind to proteins on the cell surface referred to as desmogleins, the function of which is to glue the cells together. Once the autoantibodies are bound to these proteins, the epidermal cells are no longer held together and they separate, causing blisters and sores to develop.
Symptoms
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Skin blisters sometimes join together, forming large areas of raw and very sore skin. The blisters then become crusty and start to turn into scabs. The lesions do not usually result in scarring, although areas that have become pigmented often remain so.
Diagnosis
Since this condition is so rare, patients are usually referred to a dermatologist or specialist in oral health. The initial diagnosis is based on the appearance of the mouth and skin. This is followed by a biopsy from an unbroken blister, with examination of the skin sample under a microscope. Diagnosis is then confirmed if cells appear to be separated from each other and are no longer held together. A blood sample may also be taken to measure the level of pemphigus antibody, which provides an indication of how active the condition is and influences the choice of treatment.
Treatment
These is no cure for pemphigus vulgaris, but the condition can be controlled and is more easily managed if diagnosed and treated early in the disease course. With long-term treatment, the condition can sometimes be controlled to the point that flare-ups no longer occur even after treatment is stopped. The treatments used to manage pemphigus vulgaris are similar to those used to treat severe burns.
Pemphigus vegetans
Pemphigus vegetans is a rare variant of pemphigus vulgaris that starts at an earlier age than pemphigus vulgaris. It is caused by IgG antibodies against the skin antigen desmoglein, leading to separation of the epidermal cells from each other.
Factors that exacerbate the condition include warm weather and superficial bacterial infection. The condition is characterised by cauliflower-like lesions that develop within the epidermis, breaking open to produce persistent erosions. People with this disease do not develop mouth lesions, but large areas of vegetative skin erosions that join together.
The lesions form in areas of the body where two skin areas may rub together such as the armpit or groins. Folds of skin on the neck or face may also be affected.
Pemphigus vegetans may be treated with topical and systemic antibiotics, although large growths may require surgical excision. The prognosis for patients with this condition is much more positive than for patients with pemphigus vulgaris.
International Pemphigus and Pemphigoid Foundation
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Further Reading