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Pemphigus Vulgaris

Pemphigus Vulgaris

Pemphigus is a group of rare autoimmune blistering diseases of the skin and/or mucous membranes. Pemphigus is chronic and progressive and characterised by blisters and ulcers on the skin and mucous membranes.

The oral mucosa is often the first site of involvement. Typically patients will have had multiple oral ulcers (broken blisters) that persist for weeks to months. Pemphigus Vulgaris affects the oral mucosa in nearly all cases (>80%) and affects the oral mucosa first in the majority of cases (c), and may in fact be the only site in patients.

A minority will present only with cutaneous erosions. Skin involved is typically the upper chest, back, scalp, and face, but lesions can occur on any part of the body. Note that the skin dominant form manifests the Dsg1 autoantibody profile (as in pemphigus foliaceus), whereas the mucosally dominant form manifests the Dsg3 autoantibody profile. A mixed pattern of skin and mucosa manifests a mixed Dsg1 and Dsg3 autoantibody profile. The condition progresses over weeks to months.


Skin blisters caused by pemphigus vulgaris

Sites often overlooked include around the nails (manifest as painful, red, and swollen), the pharynx and larynx (pain on swallowing and hoarseness), and the nasal cavity (nasal congestion and a bloody mucous discharge, particularly noticeable upon blowing the nose in the morning). As many as 49% of patients were shown to have laryngeal and nasal involvement (a).

Antibodies that attack one's own cells are called autoantibodies. The part of the cells that are attacked in pemphigus are proteins (desmogleins) which act as the glue that attaches adjacent skin cells, keeping the skin intact. When autoantibodies attack desmogleins, the cells become separated from each other. The skin virtually becomes unglued. This causes burn-like lesions or blisters that do not heal naturally. In some cases, these blisters can cover a significant area of the skin.

The disease appears to affect people across racial and cultural lines, so it's not possible to predict who may get pemphigus. Although fairly common in adults, pemphigus is rare in children. In children, it is most often caused by placental transmission of IgG autoantibodies in utero from a woman who has pemphigus.

If left untreated, the erosions and bullae of pemphigus vulgaris gradually spread, involving an increasing surface area, and can become complicated by severe infections and metabolic disturbances.

Before the advent of corticosteroids, pemphigus had a high fatality rate, with approximately 70+% of patients dying within a year.

With treatment, lesions can heal normally without scarring and the hyperpigmentation associated with pemphigus often resolves after several months. Most patients treated for pemphigus will enter a partial remission within 2 to 5 years. (In a longitudinal study of outcome in 40 patients with pemphigus vulgaris, 45% entered a complete and long-term remission after 5 years and 71% after 10 years.

Pemphigus vulgaris is a very serious skin disease that causes blisters. It is rare. Pemphigus vulgaris is more serious than most other blistering skin conditions. Most people with pemphigus vulgaris died before treatment became available. These days most cases can be controlled with treatment. Treatment is usually with steroid medicines and other medicines to suppress the immune system. A skin specialist should supervise treatment.

What is pemphigus vulgaris?

Pemphigus vulgaris (PV) is a rare skin condition that causes blisters. PV is a serious disease that needs treatment. If it is not treated, itis often fatal.

paraneoplastic pemphigus and Note: several skin conditions can cause blisters and it is important to know exactly which disease you benign familial pemphigus have. There are other types of pemphigus called: pemphigus foliaceous, pemphigus erythematosus,.

There are also conditions with similar sounding names that cause blisters. For example, pemphigoid. These different conditions vary greatly in their seriousness, outlook and treatment. Pemphigus vulgaris is the most common form of pemphigus and one of the most serious blistering skin conditions

Who gets pemphigus vulgaris?

PV is rare - between one and five people in a million develop PV each year in the UK. Most cases develop in people aged over 50. It is very rare in children. Men and women are equally affected. It is more common in certain groups of people - those from the Mediterranean, Indian subcontinent or of Jewish origin. PV is not infectious and you cannot catch it from an affected person.

What causes pemphigus vulgaris?

PV is an autoimmune disease. The immune system normally makes antibodies to attack bacteria, viruses. and other germs. In people with autoimmune diseases, the immune system also makes antibodies against a part of the body. In people with PV, antibodies are made against a protein that binds the skin cells together. As a result, the binding between many skin cells is broken and this causes the cells to separate from each other. Fluid builds up between the separated cells and forms blisters.

It is not known why PV and other autoimmune diseases occur. It is thought that something triggers the immune system to attack the body's own tissues. Possible triggers include viruses, infection, or other environmental factors. There may be an inherited factor which makes some people more prone to develop autoimmune diseases

What are the symptoms of pemphigus vulgaris?

�Skin blisters. Many blisters may develop on any part of the skin. The most common areas affected are the face, scalp, armpits, groins, and pressure points (buttocks, etc). The blisters can be several centimetres across and are not normally itchy. The blisters are flaccid (loose) and fragile so they easily burst. This leaves raw, red areas of skin which can be very sore and painful. The raw areas of skin heal without scarring, but the affected skin may become more pigmented (darker).
�Mouth blisters are common. The blisters burst soon after forming, to leave raw painful mouth erosions (like severe mouth ulcers). Some people with PV only get mouth blisters, without any skin blisters. Other people may get mouth blisters as the first symptom of PV.
�Other inner skin areas (mucous membranes) such as the throat, anus, genitals, vagina and front of eye are sometimes affected in a similar way to the mouth. If blisters occur in the voice box (larynx), you may get hoarseness. Sometimes blisters can occur in the gullet, which can make swallowing painful.

How does pemphigus vulgaris progress?

Often, it is the mouth blisters which develop first. These soon burst and form painful mouth erosions, which are like ulcers. The skin blisters may follow some weeks or months later.

If PV is left untreated, the blisters tend to persist and the number of blisters tends to increase. It can become distressing if there are many blisters, as they can burst to leave areas of raw, painful skin. These raw areas are at risk of infection which can be serious.

When PV is active in the mouth or throat it can become difficult to eat or drink. If this occurs you may need nutritional advice or supplements from your GP.

For More information look into: http://en.wikipedia.org/wiki/Pemphigus_vulgaris

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