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PEMPHIGUS VULGARIS

Pemphigus vulgaris (PV) is one of a group of chronic, relapsing auto-immune diseases causing blistering of the skin and mucosal membranes.

In pemphigus disorders the immune system perceives the skin and mucous membranes as foreign and an immune response is triggered.  Although it is controllable with heavy immunosuppressive treatment there is no cure available and, if left untreated, the disease can prove fatal.

PV is very rare, with most GPs never encountering the disease.  This can lead to mis-diagnosis of the condition.  Worldwide incidence is approximately 0.5-3 per 100,000 per year and onset usually occurs in middle age (4^th to 6^th decades).

 Causes

Research studies shows a genetic disposition to the disease, but although researchers have identified a variety of possible triggers, these remain speculative.  The possible triggers suggested, include: D-penicillamine derived drugs, some viruses e.g. Epstein-Barr and herpes simplex, foods from the allium family, solar irradiation and long-term stress.

Affected individuals have high concentrations of antibodies produced by the immune system.  These bind to a specific protein in the skin (desmoglein 3), causing interference with the skin’s normal function and separation of epidermal cells.  This often occurs first in the mouth, but lesions can cover a significant area of the skin.  The disease is non-infectious.

Symptoms

Someone with PV may feel ill a lot of the time, even if the disease is under control.  A patient can be either in remission or have the condition under control, but still experience some blistering lesions.  These burn-like lesions often first occur in the mouth and throat, are extremely painful, and are frequent mis-diagnosed as ulcers.  The sensation has been compared to having a candle burning in the throat.  Consequently pain control is a crucial issue.  Lesions on the skin sometimes hurt, and even if they are not painful, they often itch and burn continuously, which in itself is physically very distressing for the patient.  The pain endured by people with the condition can be very distressing, not only for the person concerned but also for carers.

Some people manage the high levels of corticosteroid and immunosuppressive drug therapies without difficulty, whilst others find that, apart from long-term side effects (eg. Osteoporosis), the daily consequences of using such drugs produce health problems which are difficult to live with.  Due to rarity, people often find it hard to get accurate and up-to-date information on the condition and most people feel extremely isolated.

Treatments

The standard treatment is with corticosteroids, which are often started at high doses (e.g. 60-100mg per day), together with immunosuppressive drugs (Azathioprine, cyclophosphamide, methotrexate).  Occasionally, cyclosporin or intramuscular gold injections are used.  Drug doses are reduced slowly to the minimum required to maintain the condition under control (which varies from individual to individual).

Topical therapies are sometimes given in conjunction with systemic drugs to help heal recalcitrant erosions.  In the pre-corticosteroid era, PV was generally fatal, and even with steroids and immunosuppressives, it remains a difficult disease to treat and has a mortality rate of about 6%.

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