The disease is caused by an autoimmune response of the organism. This means that the immune system starts to attack our own tissues including production of autoantibodies. In this case, the immune system damages the skin and subcutaneous tissues.
Damage to skin cells causes them to lose cohesion. Blisters occur in the affected areas that can be quite extensive. The blisters may perforate forming moist and painful skin ulcers. The ulcers can be easily infected by bacteria causing impetiginization. The damaged skin cover loses its barrier function and bacteria can enter subcutaneous tissue, which increases risk of severe infections including sepsis. The ulcers usually heal, but the disease has a tendency to return in repeated bouts in already healed areas.
The diagnosis is usually confirmed by a dermatologist on basis of physical examination. When uncertain, the doctors may take small samples of the affected tissue for a histological examination. There may also be a positive finding of various autoantibodies in the patient's serum.
The patients are usually treated by immunosuppressive agents, for example by corticosteroids. When the occurring wounds are infected by bacteria, antibiotic therapy is necessary. Severe bouts of the disease should be treated in an inpatient department.