Pemphigus vulgaris is a potentially serious autoimmune disorder that is characterized by painful skin lesions and oral blisters. An affected person's immune system produces antibodies that prohibit skin cells from adhering together, leading to frequent breakages and inflammation. Doctors can prescribe topical and oral medications to manage symptoms in mild cases. A serious breakout of pemphigus vulgaris typically requires hospitalization and immediate care to prevent life-threatening complications.
The exact causes of pemphigus vulgaris are not well understood, but researchers have determined that it is an inheritable condition. It is more likely to arise if a person already suffers from other autoimmune disorders. The condition may afflict a person of any age, though it is most commonly seen in patients older than 60 years of age. In rare cases, pemphigus vulgaris can be triggered by blood pressure medications or immunosuppressive drugs.
Cells in the skin and mucous membranes are bound and held together by desmoglein proteins. In the case of pemphigus vulgaris, antibodies released by the immune system travel in the bloodstream to the cells and attack desmoglein, leaving the outer layers of tissue weak. Affected skin is very delicate, and even rubbing it lightly can cause it to redden and break.
A person who suffers from pemphigus vulgaris is likely to have open sores in the mouth and blisters on the neck and torso. Severe cases often result in widespread skin problems, covering the extremities, scalp, and face. Blisters and mouth lesions can be very painful and tender to the touch, though they do not usually itch. They typically develop over the course of several days, fill with pus, and eventually rupture. Without medical attention, the condition is likely to get progressively worse and more painful.
A dermatologist can diagnose pemphigus vulgaris by evaluating the appearance of blisters and rubbing skin to see if it breaks apart. Samples of blood and skin tissue are normally collected and analyzed in a laboratory to check for specific antibodies. A patient who has mild symptoms is typically treated in the dermatologist's office with topical corticosteroids and protective bandages. He or she may be prescribed an anti-inflammatory drug and antibiotics to combat infections.
A patient who has a widespread or very painful skin problem is typically admitted into the hospital. Doctors and nurses may provide intravenous corticosteroids and fluids to help relieve immediate symptoms. Topical anesthetics are often used to ease pain in the mouth and on the skin. Blood or plasma transfusions are typically considered a final option if problems do not clear up in the hospital. With immediate treatment, home care, and frequent checkups, most patients are able to manage their conditions and lead normal lives.
