The 49-year-old woman immediately sensed something was wrong as soon as she got out of bed. Upon examination, she discovered several small blisters around her belly button, resembling the blisters one might get from wearing new shoes. Trying to alleviate the discomfort, she dressed cautiously, opting for loose-fitting clothes. However, the pain persisted throughout the day, making it difficult for her to focus on her work.
Upon returning home, she found that one of the blisters had burst, leaving behind a raw and angry red mark. Although she tried not to worry, the blisters continued to appear and worsen each day, covering her back and belly. Some blisters burst, causing clear liquid to leak out, staining her clothes and sheets.
After enduring a week of this torment, she decided to take a sick day and began searching for a dermatologist online. After several calls, she finally secured an appointment for the next day. The medical assistant who examined her immediately expressed concern as this was not a routine condition. The patient explained that her skin had been dry and itchy for months, but the blisters were a new development.
The dermatologist prescribed a cream for acne, assuming it might be the cause. However, they also considereскrкd a skin infection and prescribed antibiotics as a precautionary measure. The patient was instructed to follow up in a few weeks to monitor progress.
Unfortunately, the skin did not improve by the next appointment, and another dermatologist was consulted. They recognized the condition as a blistering illness, potentially eczema herpeticum caused by the herpes simplex virus. Along with a powerful steroid cream, an antiviral medication was prescribed and the patient was assured that this would resolve the issue.
However, in the following week, new blisters continued to appear, prompting another round of antibiotics and steroid creams. The patient’s condition worsened, with blisters covering her arms, legs, back, stomach, mouth, and scalp. Sitting was unbearable, leaving her only able to perch on the edge of a chair. Realizing a different approach was needed, the personal assistant sought out a specialist in autoimmune diseases.
Finally, through an online support group for bullous pemphigoid, the patient found a recommended specialist, Dr. Allireza Alloo. Exhausted and frustrated, she met with Dr. Alloo, who recognized her fatigue and discomfort. The patient recounted how her skin had been persistently dry and itchy, even on warm vacations. Canker sores and blisters followed, making eating difficult.
Dr. Alloo performed a thorough skin examination and concluded that the patient had pemphigus vulgaris, an autoimmune disorder. Unlike bullous pemphigoid, this disorder involves antibodies attacking the connection between cells in the upper layers of the skin, causing them to separate easily. Dr. Alloo assured the patient that she would get better.
Treatment for pemphigus often starts with steroids, but if they become ineffective or cause intolerable side effects, rituximab is the next step. This immunosuppressive drug destroys the white blood cells producing abnormal antibodies, effectively resetting the system.
The patient began her treatment with rituximab, and gradually, over the next few months, her skin started to clear up. Finally, after an arduous journey, she found relief.
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The 49-year-old woman knew as soon as she got out of bed that something was very wrong. A quick examination of her body revealed the source: around her belly button were a half-dozen blisters. They were small (the biggest ones maybe the size of a pencil eraser) and painful. They looked like the kind of blister you might get on your heel after wearing a new pair of shoes. Except they were in her belly.
He dressed carefully and chose a pair of pants that were a little baggy in the waist. She put on a long t-shirt under her sweater and hoped for the best. It was difficult to concentrate on her work: the fleshy bubbles sent out painful reminders every time she changed positions. When she got home, she immediately put on a loose-fitting dress. One of her blisters had broken, leaving a raw red mark that looked angry. She tried not to worry about it. It was September 2021; This was her first week back in the office after months of working from home, and she already had too much on her mind.
But the next day, a couple more blisters appeared. And the next day. By the end of the week, his back and her belly were dotted with a dozen of these strange bubbles. Another dozen had burst, leaving sores leaking clear liquid. At night, the open blisters soaked the gauze she applied, then the pajamas and sheets. Every movement she made opened the festering wounds that had dried, binding flesh to fabric.
After a week of this, she took a sick day and began searching for a dermatologist online; After many calls, she found an office where she could be seen the next day. The medical assistant who treated her was immediately concerned. After 40 years of practice, she had mastered the routine things…and this was not routine. The patient told the AP that her skin had been extremely dry and itchy for months, but that these blisters were new. When the AP examined the woman, she saw some intact blisters, but much of the woman’s torso was dotted with open spots. This could be acne, she said. She would give him a cream for that. But it could also be a skin infection, and for that she prescribed an antibiotic. The patient should follow up in a couple of weeks to make sure everything is going in the right direction.
They were not. At the woman’s next appointment, her skin had not improved. The AP brought in one of the dermatologists. It was clearly some kind of blistering illness, the doctor said. Possibly a type of disseminated infection called eczema herpeticum, which is caused by the herpes simplex virus. The doctor prescribed a powerful steroid cream along with an antiviral medication that she was to take for a week. That should clear things up, he told the woman confidently.
Blisters everywhere
But over the next week, the blisters and oozing spots that followed continued to appear. She was prescribed a second antibiotic. More steroid creams.
At this point, everything he did hurt. His blisters were everywhere: on his arms, his legs, and all over his back and stomach. They were even in his mouth and on his scalp. Sitting down was impossible. All she could do was sit on the edge of the chair. When she returned, the determined personal assistant brought in another dermatologist. He examined the woman closely and said, “I think this may be something called bullous pemphigoid. If so, we can treat it.”
The PA explained that bullous pemphigoid (BPE) is a rare autoimmune disease in which the body’s white blood cells create antibodies that attack the connection between the skin and underlying tissue, causing these blisters. Blood pressure is treated with high doses of steroids, and when the disease cools and blisters stop appearing, steroids can be gradually reduced and sometimes stopped completely. The disease usually resolves within a few months, but can last for years.
He started taking 60 milligrams of prednisone every day. The drug was horrible. She couldn’t sleep. She felt constantly hungry but at the same time bloated and full. She was weak. Her legs felt like they weighed 100 pounds each. But the results were surprising. There were fewer new blisters. And the raw spots that marked where old blisters had once formed began to heal. She took prednisone for two months. Her skin improved, but she gained more than 20 pounds and her weakness was so profound that she could barely get out of bed in the morning. The weight gain was depressing, but the weakness intolerable.
Seeing how disabled she was and how much her skin had improved, the personal assistant reduced her dose. New waves of blisters appeared almost immediately. The PA increased her dose, but this woman clearly needed a different approach. She needed a doctor specialized in these types of autoimmune diseases. There are groups on the Internet for BP patients, the AP told the patient. They will know the best doctors for you. She was right. Through the International Pemphigus and Bullous Pemphigoid Foundation, she found a group of peers online who had the disease and lived not far from her on Long Island. They were unanimous in recommending her: Dr. Allireza Alloo, an associate professor and attending physician at Hofstra/Northwell’s Zucker School of Medicine.
Press the reset button
When Alloo walked into the exam room to meet his new patient, he could see how tired she was. And frustrated. She had been uncomfortable with herself for months and the treatment was almost as bad as the illness. She started itching, she reported. She always suffered from dry skin in the freezing depths of winter, but a couple of years earlier her skin had the same dry itch while she was on vacation with friends in warm Hawaii. She bought her usual winter creams (moisturizers and low-dose steroid creams) and slathered on at regular intervals. She helped me, but the itching never completely went away.
Then he started getting canker sores, huge and painful. It hurt me to eat. When one burst in her mouth, she had to limit her diet to soups and smoothies. And then her blisters started.
Alloo asked her to put on a gown and then gave her a complete skin examination. Her blisters ranged from the size of a BB pellet to the size of a quarter. She could push them down and they would not break or spread outward, as might be seen with bullous pemphigoid. The skin covering these blisters was thin, almost translucent. And they were delicate. Alloo could understand why early dermatologists had thought he had PA. This disease usually begins with intense itching before blisters appear. But the damaged tissue is deep, so the skin that forms the blisters is thicker. And the blisters themselves are tight and not soft like these. This was not BP
“You have pemphigus vulgaris,” Alloo told him, “and you are going to get better.” Pemphigus gets its name from the Greek word for blister. It was first described in the 18th century, and until the 20th century, with all its advances, any disease that caused blisters (and there are many) was called pemphigus. Pemphigus vulgaris is an autoimmune disorder, like BP, but even rarer. In this disease, antibodies attack the connection between cells in the upper layers of the skin so that it separates easily from the lower layers. An old test for the disease, called the Nikolsky test, involved rubbing normal-looking skin next to the blisters. In pemphigus, that skin often peels off in thin sheets. Mouth sores are usually the first symptom of the disorder. Until recently, the diagnosis was made only by biopsy. Now a blood test can help identify the specific antibodies causing the damage.
As with blood pressure, Alloo explained to the patient, pemphigus can often be treated with steroids. But when these are not enough or cause intolerable side effects, the next step is usually a medication called rituximab. This powerful immunosuppressive drug destroys antibody-producing white blood cells. When the next generation of these cells is created, they no longer produce abnormal antibodies. It’s like hitting the reset button on a cellular level.
The patient received her first two doses of rituximab shortly thereafter. Over the next few months her skin began to clear up. It’s been a year and a half since she started treatment and she still hasn’t fully recovered; She expects to need a couple more doses of rituximab. But she trusts that Alloo was right. She will recover again.
Lisa Sanders, MD, is a contributing writer for the magazine. Her latest book is “Diagnosis: Solving the Most Perplexing Medical Mysteries.” If you have a solved case to share, please write to her at Lisa.Sandersmdnyt@gmail.com.
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