Pemphigus Vulgaris

Vulg1
Vulg2

What is it?
Pemphigus vulgaris (PV) is a rare condition, severe and potentially life threatening if left untreated. It is characterised by flaccid (loose) blisters of the skin and mouth and after the blisters rupture by widespread painful erosions.

Most patients develop the mouth lesions first. Shearing stresses on normal skin can cause new erosions to form.

What are the symptoms and how does the disease progress?
It begins with mouth ulcers which can often be mistaken for others illnesses and this is then followed by skin blisters.

What causes it?
PV is an autoimmune disease which results in specific skin cells which fall apart. In autoimmune disease the antibodies which normally attack foreign bodies end up attacking the individuals own cells. In PV the immune system attacks a protein which binds cells together which results in cells separating from each other. Fluid builds up between the separated cells and then forms the blisters. The cause of this autoimmune disease is unknown.

How can it be diagnosed?
If PV is likely from original observations then a biopsy and of the skin is taken. A  blood test can also be carried out to locate the culprit antibodies. The level of auto-antibody can give a good indication as to how active the disease is. This can also be measured from time to time to indicate the effectiveness of treatment.

What is the treatment process?
Patients are treated by dermatologists. Severe cases need very high doses of systemic steroids and once the disease appears to be under control the medication is altered accordingly.

Complications
Patients can have a terrible time with weight gain and other side effects from systemic corticosteroids and the immunosupressants needed to control the disease.  Also, patients can suffer from the lesions which resist healing.  Side effects are the leading cause of death. Infections of all types are common as the continuous bursting of blisters reveals raw skin that is vulnerable to infection.  When PV is active in the mouth the patient will find eating and drinking very difficult.

About one third of patients will go into complete remission within 3 yrs.

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The prevalence of skin disease exceeds that of obesity, hypertension, or cancer. Despite skin being the largest organ of the human body, dermatological research remains one of the most under funded areas of medicine. In a world where society has an increasing preoccupation with image and it’s importance to every aspect of a person’s life, sufferers of skin diseases are feeling and being more marginalised and isolated than ever.

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