Dry, itchy skin becomes painful blisters all over

According to the patient, she had always had dry skin during the winter, but a few years earlier, she began to experience the same dry itchiness even in warmer climates. Although she used moisturizers and low-dose steroid creams on her skin, the itch never completely went away.

Then, she started to get large and painful canker sores, making it difficult for her to eat. After one erupted in her mouth, she limited her diet to soups and shakes.

After that, another strange symptom appeared: blisters. The first blisters appeared around her belly button. They were small, but painful, and more would soon appear on her back and stomach. Many of these blisters also ruptured, leaving open sores.

When the patient first visited a dermatologist, the physician assistant (PA) who examined her said the blisters might either be acne or a skin infection. The patient was given an acne cream, as well as an antibiotic prescription, and told to follow up in a couple weeks.

However, at the patient's next appointment, her skin still hadn't improved. This time, the PA brought in one of the dermatologists who said that she might have eczema herpeticum, a type of disseminated infection caused by the herpes simplex virus. The patient was prescribed a potent steroid cream and an antiviral medication.

This new round of medication didn't help either. Even when she was prescribed more steroid creams and antibiotics, her blisters didn't go away. They had spread all over her body, including in her mouth and on her scalp.

An autoimmune condition was behind the blisters — but which one?

The patient visited the dermatologist again, and the PA brought in a different doctor to examine her. This time, the doctor diagnosed the patient with a condition called bullous pemphigoid (BP).

BP "is a rare autoimmune disease in which the body's white blood cells create antibodies that attack the connection between the skin and the tissue below," which then causes blisters, Sanders writes. The condition is treated with high-dose steroids, which can be tapered off and sometimes completely stopped depending on how well the symptoms resolve. Treatment can take a few months but may last for years.

After the diagnosis, the patient began taking 60 milligrams of prednisone every day. The medication reduced the number of new blisters and helped heal her skin, but the patient also experienced significant side effects, such as weight gain and weakness.

Taking into account her skin improvement and side effects, the PA lowered the patient's dose of prednisone. However, her skin immediately began worsening again. A new approach was needed, and the PA suggested the patient find a doctor specializing in these kinds of autoimmune disorders through online BP groups.

Through the International Pemphigus and Pemphigoid Foundation, the patient was recommended to Allireza Alloo, an associate professor and attending physician at the Zucker School of Medicine at Hofstra University and Northwell Health.

By the time the patient met with Alloo, she "had been uncomfortable in her own skin for months, and the treatment was almost as bad as the disease," Sanders writes. The patient explained the progression of her symptoms before Alloo examined her.

After doing a full exam of the patient's skin, Alloo saw that her blisters were thin and delicate, not tense and deep like the blisters commonly seen in BP. This helped him realize that she had another condition altogether.

"You have pemphigus vulgaris," Alloo told the patient, "and you are going to get better."

Like BP, pemphigus vulgaris is an autoimmune disorder, but it is even rarer. "In this disease, antibodies attack the connection between cells in the topmost layers of the skin so that it is easily separated from the layers below," Sanders writes. "… Until recently the diagnosis was made solely by biopsy. Now a blood test can help identify the specific antibodies that do the damage."

Pemphigus vulgaris is usually treated with steroids, but when they are not effective or cause significant side effects, another medication called rituximab is recommended. Rituximab is an immune-suppressing drug that destroys antibody-producing white blood cells so that the next generation of these cells don't create abnormal antibodies.

After receiving two doses of rituximab, the patient's skin began to clear, but even a year and half later, she is still not fully recovered. "[S]he expects she'll need a couple of more doses of rituximab," Sanders writes. "But she is confident Alloo was right. She is going to get well again." (Sanders, New York Times Magazine, 10/7)

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