Pemphigus Vulgaris - Causes, Symptoms and Treatment

Definition

Pemphigus vulgaris is a rare auto-immune disease of skin. Pemphigus is derived from the Greek word pemphix meaning bubble or blister. Pemphigus describes a group of chronic bullous diseases, originally named by Wichman in 1791. The term pemphigus once included most bullous eruptions of the skin, but diagnostic tests have improved, and bullous diseases have been reclassified. This is an informational page for the patients and their families and it contains links which they may find useful. It does not contain medical advice. Affected individuals have high concentrations of antibodies produced by the immune system. These bind to a specific protein in the skin causing interference with the skin’s normal function. The cells no longer stick together and become separated from each other. Pemphigus is uncommon. It occurs almost exclusively in middle-aged or older people of all races and ethnic groups. About one-half of the cases of pemphigus vulgaris begin with blisters in the mouth, followed by skin blisters.

Causes

1. It is an autoimmune disorder in which the immune system produces antibodies against specific proteins in the skin and mucous membrane. These antibodies produce a reaction that leads to a separation of epidermal cells.
2. The exact cause of the development of antibodies against the body's own tissues is unknown.
3. Sometimes pemphigus vulgaris appears in reaction to medications, although this is rare. Pemphigus may be a side effect of certain blood pressure medications or chelating agents.
4. It is caused at the age above than 50. So age is also a decisive factor in this disease.

Symptoms

1. Pemphigus vulgaris usually begins with blisters in your mouth, which then erupt on your skin.
2. The blisters typically are painful, but don't itch.
3. Blisters present on the mucous membranes of your genitals can break out.
4. Pemphigus is uncommon. It occurs almost exclusively in middle-aged or older people of all races and ethnic groups.
5. The blisters are relatively asymptomatic, but the lesions become widespread and complications develop rapidly and may be debilitating or fatal.
6. The primary lesions are flaccid bullae of various sizes, but often skin or mucosa just shears off, leaving painful erosions.

Treatment

1. Patients may continue to experience mild disease activity while under optimal treatment.
2. Treatment with immunosuppressives is standard therapy. Prednisone in addition other immunosuppressives may be needed. The prednisone dosage is dropped as rapidly as possible once the other immunosuppressive begins to have an effect, typically 2- 3 months after starting treatment.
3. Treatment may require hospitalization, including care in a burn unit or intensive care unit.
4. Any evidence of infection should, after culture, be treated aggressively with the appropriate antibiotic.
5. Effective psychological support is needed as it's easy for anyone with a chronic illness to develop depression.
6. 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.