Dr. Patton, who is now chief of Dermatology at UPMC Shadyside, finds it “intellectually challenging and rewarding to care for patients like Monica.
“When I was in medical school, what interested me most was studying rare, life-threatening skin diseases that required aggressive therapies,” he says. “Pemphigus is one of those. It is an immunobullous, or blistering, disease. While we do not know its exact cause, it is a condition in which the body will make antibodies that attack different proteins in the skin. Before the discovery of corticosteroids, pemphigus was 100 percent fatal. Essentially, patients just lose the function of the skin, because it winds up being replaced with these blisters and erosions. They become susceptible to infection.”
Because Ms. Abbott’s pemphigus had gone unrecognized for so long, it was particularly challenging to treat. She was hospitalized for more than three months, and controlling her symptoms took more than a year. She battled pain and exhaustion as flare-ups returned again and again. Many people thought she could not survive. She had to go to a burn unit for treatment of her blisters. She developed infections and a blood clot. Her bones deteriorated. She underwent apheresis, a procedure used to destroy harmful antibodies by removing blood, separating it into plasma and cells, and reintroducing the cells. She lost some of her hair. Even her vocal cords were affected.
“Although therapies are much better today, pemphigus may require long-term treatment to keep it in remission,” says Dr. Patton, who now directs the UPMC Center of Immunobullous Diseases. Patients travel long distances for care at this center of excellence, where treatment is aimed at reducing symptoms and preventing complications. It may include the use of corticosteroids, immunosuppressive drugs, and more recently immunotherapy.