Type 1 diabetes, rheumatoid arthritis and lupus are familiar autoimmune diseases; but it's the rare endemic pemphigus foliaceus which plagues nearly 5 percent of men age 30 to 70 and a smaller percentage of postmenopausal women living on the outskirts of the tiny municipality of El Bagre, Colombia providing a rare opportunity to dissect the factors that prompt the immune system to harm instead of protect.
"The cells of the patients, they will tell us," says Dr. Ana Maria Abreu-Velez, dermatologist, immunologist and a native of the country where the disease surfaced in the mid-1980s as a mining boon changed the landscape. "These people were dying. They had huge blisters, their skin was coming apart," she says of the disease in which the immune system attacks multiple proteins that hold skin cells together. The disease can be limited to patches on the skin but its more deadly systemic version can spread across the skin and, preliminary data indicates, to other organs such as the heart and brain.
In 1990, Dr. Abreu-Velez was living less than an hour's flying time from El Bagre when she first made the trip through 33 creeks and the jungle to get to the town. The disease was emerging as those jungles were cleared to get to the gold in the rivers and rocks. That journey changed her life and the lives of patients with the disease, who were shunned by townspeople and even family members concerned about black magic and contagion. Victims congregated on the outskirts of town, untreated and dying. Dr. Abreu-Velez helped local medical doctors called shaman and townspeople understand that the disease was not contagious and establish programs and laws to make food and health care available.