Pemphigus is a rare autoimmune disease that causes painful blisters on the skin and mucous membranes.

PemphigusPemphigus usually appears on the mucous membranes, which are in the mouth, throat, nose, eyes, lungs or genitals. Most frequently the disease starts by blistering in the mouth and the skin.

This illness can be dangerous, and its treatment is necessary – requires the use of corticosteroids, which task is to suppress the immune system. Untreated disease can lead to numerous complications, including death.

The symptoms of pemphigus

Among the most characteristic symptoms, it should be distinguished painful blisters, which appear in the area of the mouth or skin, blisters near the surface of the skin which then turn into scabs or peels in place of the bladder.

There are several types of pemphigus depending on this where the appearance of skin lesions is. Besides the usual type, should be mention a pemphigus foliaceus – appearing in the mouth, on the skin of the face and head and then on the chest and back, pemphigus vegetans with blisters in the groin area, armpits and legs as well as pemphigus paraneoplastic – an extremely rare, that appears in those affected by certain cancers.

Causes of pemphigus

When the immune system produces proteins, known as antibodies, their function is the disposal of foreign substances such as bacteria or viruses. In the case of pemphigus, the situation is slightly different – immune system wrongly produces antibodies against the proteins in the healthy skin and mucous membrane, which results in the appearance of blisters.mikroskop3

The antibodies in the case of autoimmune disease break a bond between healthy cells, which accumulates liquid between the layers of the skin and then blisters and skin problems occur. The exact cause of pemphigus is not discovered, yet.

Very rarely, it happens that certain kinds of drugs may cause pemphigus. Among them, may be mentioned penicillamine – which is a chelating agent that removes some of the materials – so-called ACE-inhibitors from the blood. It belongs to the drugs for blood pressure.

Pemphigus is not infectious, so you can not catch it from any other person. Has not been proven unequivocally also on whether it can be passed on genetically, but the fact of the appearance of the disease in immediate family members may increase the risk of its occurrence.

Treatment of pemphigus

After diagnosing the type of pemphigus, the treatment is mainly aimed at reducing pain and symptoms and the prevention of complications such as infections. It includes one or more methods and medicines:

  • corticosteroids, and immunosuppressants – unfortunately, they have a lot of side effects like an increased risk of osteoporosis, cataracts, glaucoma, increasing blood sugar levels, loss of muscle mass, gastric ulcer or water retention;
  • supplementation of calcium and vitamin D, low sugar diet, medications to support the prevention of side effects;
  • corticosteroid creams for application directly to blisters;
  • medicines that suppress the immune system as azathioprine, methotrexate, mycophenolate mofetil, cyclophosphamide, rituximab;
  • antibiotics, antifungals, and antivirals;
  • in extremely serious cases, and pain while eating is used intravenous feeding;
  • plasmapheresis – removal of antibodies attacking the skin in a particularly difficult case;
  • hospital wound healing – similar to that used in the treatment of severe burns.

Among the treatments, there are also anesthetic lozenges blisters in the mouth, soothing balm, wet dressings, painkillers, soft diet, avoiding sun exposure and others.ampulki

Complications of the disease may include a plurality of skin infections, sepsis and spread of infection via the blood, dehydration, and a plurality of side effects from drugs.

Pemphigus is a chronic disease that can not be cured. Remission and improvement occur, very often, as a result of corticosteroid treatment. Blisters heal slowly – especially those within the mouth. It takes usually six to eight weeks but sometimes completes healing lasts for years. Some people need regular doses of medication for life.

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