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Pemphigus Vulgaris - Causes, Symptoms and Treatment


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Pemphigus vulgaris is a rare auto-immune disease of skin. It is generally caused by auto antibodies directed against critical adhesion molecules of squamous epithelial cells, the desmogleins. The blisters, are usually seen in the mouth which eventually rupture and become painful ulcers. Blisters break easily and are slow to heal results from the action of autoimmune antibodies which are localize to intercellular sites of stratified squamous epithelium. Pemphigus vulgaris occurs almost exclusively in middle-aged or older people of all races and ethnic groups. Most patients first present with lesions on the mucous membranes such as the mouth and genitals. Several months' later blisters on the skin may develop or in some cases mucosal lesions are the only manifestation of the disease. Sometimes people do not realise that there are several different diseases with similar sounding names to pemphigus vulgaris. Pemphigoid and benign familial pemphigus are completely different diseases from pemphigus vulgaris.

Pemphigus vulgaris is the most frequently diagnosed form of pemphigus. Sores and blisters usually start in the mouth. The sores don't always look like blisters. 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 racial groups. Pemphigus vulgaris should be suspected in any bullous disorder or chronic mucosal ulceration. Pemphigoid and benign familial pemphigus are completely different diseases from pemphigus vulgaris. The primary aim of treatment is to decrease blister formation, prevent infections and promote healing of blisters and erosions. Oral corticosteroids are the mainstay of medical treatment for controlling the disease. Plasmapheresis is a process whereby antibody-containing plasma is removed from the blood and replaced with intravenous fluids or donated plasma. Plasmapheresis may be used in addition to the systemic medications to reduce the amount of antibodies in the bloodstream.

Causes of Pemphigus vulgaris

Pemphigus vulgaris is an autoimmune disorder which generally causes blistering. This disease is not contagious that is not transferable by touch. The disease may be a result of reactions to medications but this rarely occurs. This may be an adverse reaction to thiol groups contained by most of the drugs and the Alliums family, which includes garlic, onions and leeks.

Common causes and risk factors of Pemphigus vulgaris:

  • Certain blood pressure medications.

Signs and Symptoms of Pemphigus vulgaris

Many blisters may develop on any part of the skin. The most common areas affected are the face, scalp, armpits, groins, and pressure points. The primary lesions are flaccid bullae of various sizes, but often skin or mucosa just shears off, leaving painful erosions. Lesions also may occur in the upper esophagus. Cutaneous bullae typically arise from normal-appearing skin, rupture, and leave a raw area and crusting. Itching is usually absent. Open skin lesions often become infected. If large portions of the body are affected, fluid and electrolyte loss may be significant.

Sign and symptoms may include the following :

  • Mouth or skin ulcers.
  • Recurrent or relapsing.
  • Oozing may also occur.
  • Peeling superficially or detaching easily.
  • Draining, oozing, crusting.

Treatment for Pemphigus vulgaris

The primary aim of treatment is to decrease blister formation, prevent infections and promote healing of blisters and erosions. Oral corticosteroids are the mainstay of medical treatment for controlling the disease. Some antibiotics are also effective, particularly minocycline and doxycycline. Intravenous immunoglobulin (IVIg) is occasionally used. Intravenous fluids, electrolytes, and proteins may be required. Mouth ulcers, if severe, may mean intravenous feeding is needed. Anesthetic mouth lozenges may reduce the pain of mild to moderate mouth ulcers. Localized treatment of ulcers and blisters may include soothing or drying lotions, wet dressings, or similar measures.

Treatment may include:

  • Some immunosuppressive drugs such as Azathioprine, Dapsone, Tetracyclines, Cyclophosphamide, Methotrexate are may be used.
  • Intravenous fluids, electrolytes and proteins may be required.
  • Treatment which is generally used is the administration of oral steroids, especially prednisone.
  • The high doses of Corticosteroids are given in the form of tablet or injection to effectively treat pemphigus vulgari.
  • Treatment may require hospitalization, including care in a burn unit or intensive care unit.
  • This disease may benefit from avoiding foods, such as spicy foods, tomatoes, orange juice, and hard foods that may traumatize the oral epithelium mechanically, such as nuts, chips, and hard vegetables and fruit.