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Pemphigus - Pictures, Symptoms, Treatment, Causes

Updated on December 27, 2013

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What is Pemphigus?

Pemphigus is a group of rare autoimmune disorder involving the skin and the mucous membrane and is marked by the development of non-healing blisters. The blister is the result of the auto-antibodies attacking a certain protein called desmoglein.

Desmoglein involved in pemphigus is a protein responsible for attaching together the cells through the desmoses formed by the group of protein in desmoglein. The protein group of desmoglein is the one being attacked by the antibodies in pemphigus resulting to separation of cells in the mucous membrane and skin that leads to formation of blisters.

Pemphigus can occur to people at any age without racial and sexual predilection although the peak onset is usually in middle age or in older people. The disorder can be potentially fatal although mortality is related to complication of infection resulting from treatment applied in suppressing the immune system. The prognosis in older patient is relatively poor as linked to weak immunity and treatment in suppression of the immune system.

Symptoms

Pemphigus is divided into three main types classified according to their clinical and immunological manifestation.

Pemphigus vulgaris is the most common type of pemphigus occurring in about 70% of reported cases. Blisters initially develop in the oral cavity characterized as painful sores. The blisters later on erupt and spread later on in the throat. The onset of painful blisters in the mouth and the throat accounts for the difficulty to eat and swallow. Pemphigus vulgaris can occur at any age although it most likely to strike people between the ages of 40 to 60 years without sexual and racial predilection although it is predominant in Ashkenazi Jews. Painful blisters in this type of pemphigus can also spread to the skin while Nikolsky’s sign is noted when a firm pressure is applied in area of the skin unaffected by blisters.

Pemphigus foliaceus is fewer in incidence as compared with pemphigus vulgaris and is linked with the attack of antibodies in Desmoglein 1 or DSG1. This type of pemphigus does not involve the oral cavity and the blisters initially develop on the scalp and face which may then spread to the chest and the back. The sore in this type of pemphigus is rather dry and crusty and often itchy although not as painful and less in severity among the other types of pemphigus.

Paraneoplastic pemphigus is the most severe and serious form although rare type of pemphigus. The painful blisters in this type of pemphigus forms both in the mouth and skin including the lips and throat and can also affect the lungs. Paraneoplastic pemphigus develops as secondary to an existing cancer or neoplasm. It is usually found as complication, although rare, in patient suffering from cancer of lymphoma and Castleman’s disease. The involvement of lungs in this type of pemphigus often results to constrictive bronchiolitis.

Pemphigus is a rare skin disorder that affects the mucous membrane and the skin and the general symptoms include the following:

Mucous membrane

  • Blisters or lesions in the mouth are painful and often heal very slowly
  • Bullae in the mouth are intact with lesions that are poorly defined
  • Difficulty in eating and swallowing.

Skin

  • Blisters on skin are rather loose than intact
  • Extension of lesions to other areas of the skin
  • Lesions are increasing in size
  • Area of the affected skin is painful although not itchy
  • Lesions may affect the nails.

Causes

The exact cause of pemphigus remains vague and hereditary factor is not clear as well. Pemphigus is believed a disorder of the autoimmune system where the antibodies mistakenly attack the healthy cells in the body. The auto-antibodies especially attack o the group of protein called desmoglein which is responsible for producing desmoses that binds the cell of the skin and mucous membrane together. The attack o this group of protein leads to separation of cells allowing for fluid to collect in between cells of the skin thereby forming blisters which mark the disorder.

The exact trigger for the onset of any type of pemphigus has yet to be identified although factors are being considered which include the following:

  • Age factor where older people are more at risk for pemphigus
  • People with weakened and deficient immune system
  • Prolonged exposure to ultraviolet rays
  • Certain medication especially those used for high blood pressure and chelating agents
  • People suffering from cancer.

Treatment

The aim in treating pemphigus is in reduction of formation of blisters while promoting healing and preventing complications. The mode of treatment depends on the type of pemphigus and underlying condition contributing to its onset and the overall health status of the patient. Treatment is generally in suppression of the auto-antibody and inflammatory response of the body.

Pharmacotherapy includes the use of corticosteroid for managing inflammation and drugs in suppressing the immune system such azathioprine and methotrexate. Antibiotics and other drugs use for preventing and treating infections are also prescribed.

Pemphigus that is rather widespread requires a hospital stay to monitor and facilitate stabilization of fluid and electrolyte while treating and protecting patient highly vulnerable to infections and other medical complications.

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      Daniele 2 years ago

      Really wish you had been around when I was prangnet! I tried to get my husband to take some pics of me but he didn't quite get it. Now there's no getting that time back! Great pics!