Rare autoimmune disorder targets skin in aging populations
Dear Doctors: I am 75 years old, and two years ago, I was diagnosed with bullous pemphigoid. I understand this is an autoimmune problem. I also understand that this is more common in older people. I would like to know more about it. I have been prescribed minocycline, which has been helping.
Dear Reader: You are correct that bullous pemphigoid is an autoimmune disorder. That’s a condition in which certain cells in the immune system mistakenly identify the body’s own tissues as a threat. This causes them to go on the attack, which damages and destroys the tissues that they target. In the case of bullous pemphigoid, the immune system attacks the proteins that act as the glue between the two main layers of the skin. These are the epidermis, which is the outer layer of skin, and the dermis, the lower layer of skin that houses blood vessels, sweat and oil glands, nerves, hair follicles, connective tissue and other structures.
The initial symptom of bullous pemphigoid is often an itchy rash, which typically appears on the torso or the limbs. The inflammation that arises due to the immune cells’ rogue activity causes the skin to turn red. The areas of rash also become raised and swollen, as happens when someone has hives. In some cases, as bullous pemphigoid progresses, watery blisters begin to form on the surface of the skin. These can be numerous and can grow quite large. In some cases, they may contain blood. It’s possible for the condition to affect the mucous membranes, including those in the eyes, mouth, esophagus and genitals, but this is rare.
The causes of bullous pemphigoid, which typically develops at age 70 and older, are not fully understood. There is evidence that in some people, it may be triggered by medications. These include certain diuretics, antibiotics, blood pressure drugs and nonsteroidal anti-inflammatories. Again, the reasons for this response by the immune system are not yet known, but a genetic predisposition is suspected to play a role. The condition is somewhat rare, but cases have recently begun to increase. This may be due to the growing population of older adults who, with a wide range of health conditions, are exposed to numerous drugs. It may also be that growing awareness of the condition has led to an increase in diagnoses.
The goal of treatment is to limit the development of new lesions, promote skin healing, manage symptoms and prevent complications. Treatment plans are tailored to each patient’s specific case of bullous pemphigoid. They take into account the individual’s general health, as well as any preexisting conditions. Medications include systemic or topical steroids to manage inflammation and itching, antibiotics for infection and immunosuppressive drugs to calm the immune response.
Minocycline, which you are finding helpful, is an antibiotic used for bacterial infections. Episodes of bullous pemphigoid are often cyclic. They develop, peak, gradually recede and then return. The data shows that with treatment, the disease can go into remission. For about half of patients, this occurs after about two years. To remain in remission, continued treatment may be necessary.
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