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Extreme itching—A downhill experience
Jones S29
J AM ACAD DERMATOL VOLUME 51, NUMBER 1
Extreme itching—A downhill experience Rory Jones New York, New York
T
he intensely itchy sores that began on my hands—and eventually covered my face, head, and arms—first appeared almost six years before I was diagnosed with dermatitis herpetiformis (DH). The road to diagnosis, and eventually to control, was long and difficult, profoundly affecting me, my family, and my concept of what constitutes ‘‘illness.’’ I am a writer and producer of educational films and new media and very involved with my family and various charities. I enjoy the heavy schedule and constant activity. I recharge between projects and get going on the next. The symptoms of what I now know to be celiac disease probably began in 1982, after my daughter was born. My thyroid stopped working and I was constantly tired. Both were attributed to the pregnancy and treated with Synthroid (levothyroxine). In 1993, I was diagnosed with osteoporosis. The early onset was never questioned, and I was put on Fosamax (alendronate). But by then, my constant exhaustion and growing joint pain led to numerous doctor visits. I was tested for arthritis, low-grade infections, and a list of other conditions that matched my symptoms. All negative. I was told that there was nothing wrong with me. Perhaps I was over-stressed. I also developed itchy sores on my hands. I treated them with over-the-counter creams, but our family mantra was soon ‘‘stop scratching, you’ll get scars.’’ By 1995, the sores appeared regularly on my hands and fingers—always mirrored left to right— and the itching became intense. When they developed on my eyelids and forehead, I went to a dermatologist. He examined my skin and treated the sores with various cortisone creams. The diagnosis was ‘‘unexplained dermatitis,’’ possibly from dry skin and/or stress. They were not malignant, so I was told there was nothing to worry about. Over the next few months, as my joint pain increased, I started to take Advil (ibuprofen) regularly and the sores spread up my arms, and my face flared up more
0190-9622/$30.00 ª 2004 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2004.01.020
often. Occasionally, sores appeared on my legs. The facial sores would form hard crusts before they peeled off—my so-called ‘‘iguana period.’’ I could find no food or environmental pattern that coincided with the flares. I used the creams as needed, learned to live with the discomfort, and got on with my life. Over the next three years, I visited my internist regularly and reported the almost constant joint pain, overwhelming fatigue, headaches, sores, and itching. I had complete work-ups, visited numerous specialists, had many tests and everything was always ‘‘normal.’’ I had scratch tests for most of the usual food and airborne suspects. All negative. My psychological state was explored during many of these exams and I tried to explain that I was stressed and depressed because I felt sick all the time, not the other way around. In 1997, a new endocrinologist I was referred to at Columbia/Presbyterian was the first person to note that the severity of my osteoporosis and early onset was definitely not ‘‘normal,’’ and ordered more tests to determine an underlying cause. That year, I wore sunglasses to meetings when my eyes crusted and long sleeved shirts to cover my arms. In the summer, my friends thought I was being overly cautious about sun exposure, an excuse for which I was grateful. I did not mention that sunscreen burned the sores I was now constantly hiding. The dermatologist warned me about using too much cortisone cream, especially on my face, but it was the only thing that controlled the itching. At times, the itching would wake me up at night—I wanted to scratch my bones. By 1998, after trying cold compresses, yogurt, and anything else suggested, I used more and more topicals, took more nonsteroidal anti-inflammatory drugs, and became impossibly short tempered. My family bore the brunt of my distress. My husband, who had been saintly throughout all this, insisted I do ‘‘something, anything’’—see different doctors, take a break, find a way to ‘‘pull myself together.’’ I found it difficult to rationalize the gravity of the problem—people do not itch to death, so this needed to be put into perspective. I was told repeatedly by medical professionals that there was nothing wrong with me. So I stopped working and focused on rest, diet, and exercise. After three months of relaxed,
S30 Jones
healthy living, I felt worse than ever and hit a real physical and mental low. Luckily, the endocrinologist had sent a blood sample of mine to Peter Green, MD, at Columbia who was doing a random screening for celiac disease in osteoporosis patients and I was told that I had tested positive. Knowing little about the disease, I was scheduled for an endoscopy, which surprised me since I had no gastrointestinal symptoms. At the hospital, while Dr. Green explained the procedure, he saw me scratching my hands and face. Examining the sores, he asked if anyone had mentioned dermatitis herpetiformis to me? Would I have a biopsy? The endoscopy results were inconclusive—the mucosa was partly damaged, partly normal. The skin biopsy was not—I had dermatitis herpetiformis. The diagnosis dramatically changed my life. On a gluten-free diet, the joint pain and exhaustion started to fade within six to eight weeks. In three months, I felt better and had more energy than I had had in years. The clouds disappeared. Unfortunately, getting the DH under control was far more difficult. I found a dermatologist in New York who specialized in DH. She did not feel it necessary to put me on dapsone if I could control the sores through my diet. My skin continued to erupt, but within six months was dramatically better and the outbreaks were less severe and extensive. I saw her twice and seemed to be doing well on a strict gluten-free diet with topicals to control the occasional itching. I was again warned about cortisone use—but it was not easy living in my itchy skin when I had a flare-up. The dapsone alternative was not without its own dangers. While the outbreaks were much less severe, it was a roller-coaster ride to determine what was causing them. I was super-sensitive to accidental gluten ingestion, and found that iodine also seemed to flare up the DH. Nevertheless, my skin continued to improve. Every six months was better than the last.
J AM ACAD DERMATOL JULY 2004
Then, in mid-2001, I developed an almost constant red rash on parts of my face. My DH specialist was no longer in practice and I was referred to a dermatologist who diagnosed rosacea. She prescribed creams and scrubs in addition to the cortisone. When one area of my cheek started to bleed, I was referred to yet another dermatologist who took one look at me and said I was having a cortisone reaction and needed to stop using it on my face immediately. I was put on Protopic (tacrolimus) and went through a rough six weeks, but stopping the cortisone eventually eliminated the rash. The final chapter, or perhaps coda, to the story occurred in early 2003. I developed sores all over my arms and hands that looked and itched like DH, although they were not always mirrored. I was (and am) following a strict gluten-free diet, but I also began taking dapsone. A month later, I had a systemic allergic reaction to the drug. On re-examining my arms, the dermatologist noted that there are eczemas that are almost identical to DH—which is probably exactly what I had developed. Tacrolimus is now the cream of choice. I have not used the fluocinonide. I keep it for itching emergencies. The lack of knowledge about DH and the emphasis on treating symptoms rather than exploring causes made my journey to diagnosis a disturbing and painful one. I was given creams that covered up the problem and created others. Nevertheless, I consider the DH diagnosis one of the best things that has happened in my adult life—ironically, it took a gastroenterologist to see it. My symptoms finally had a name and one voice—I only wish it had spoken sooner. For more information, please visit the Celiac Disease Center at Columbia Web site at www. celiacdiseasecenter.columbia.edu or the Gluten Intolerance Group Web site at www.gluten.net.
J AM ACAD DERMATOL VOLUME 51, NUMBER 1
Extreme itching—A downhill experience Rory Jones New York, New York
T
he intensely itchy sores that began on my hands—and eventually covered my face, head, and arms—first appeared almost six years before I was diagnosed with dermatitis herpetiformis (DH). The road to diagnosis, and eventually to control, was long and difficult, profoundly affecting me, my family, and my concept of what constitutes ‘‘illness.’’ I am a writer and producer of educational films and new media and very involved with my family and various charities. I enjoy the heavy schedule and constant activity. I recharge between projects and get going on the next. The symptoms of what I now know to be celiac disease probably began in 1982, after my daughter was born. My thyroid stopped working and I was constantly tired. Both were attributed to the pregnancy and treated with Synthroid (levothyroxine). In 1993, I was diagnosed with osteoporosis. The early onset was never questioned, and I was put on Fosamax (alendronate). But by then, my constant exhaustion and growing joint pain led to numerous doctor visits. I was tested for arthritis, low-grade infections, and a list of other conditions that matched my symptoms. All negative. I was told that there was nothing wrong with me. Perhaps I was over-stressed. I also developed itchy sores on my hands. I treated them with over-the-counter creams, but our family mantra was soon ‘‘stop scratching, you’ll get scars.’’ By 1995, the sores appeared regularly on my hands and fingers—always mirrored left to right— and the itching became intense. When they developed on my eyelids and forehead, I went to a dermatologist. He examined my skin and treated the sores with various cortisone creams. The diagnosis was ‘‘unexplained dermatitis,’’ possibly from dry skin and/or stress. They were not malignant, so I was told there was nothing to worry about. Over the next few months, as my joint pain increased, I started to take Advil (ibuprofen) regularly and the sores spread up my arms, and my face flared up more
0190-9622/$30.00 ª 2004 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2004.01.020
often. Occasionally, sores appeared on my legs. The facial sores would form hard crusts before they peeled off—my so-called ‘‘iguana period.’’ I could find no food or environmental pattern that coincided with the flares. I used the creams as needed, learned to live with the discomfort, and got on with my life. Over the next three years, I visited my internist regularly and reported the almost constant joint pain, overwhelming fatigue, headaches, sores, and itching. I had complete work-ups, visited numerous specialists, had many tests and everything was always ‘‘normal.’’ I had scratch tests for most of the usual food and airborne suspects. All negative. My psychological state was explored during many of these exams and I tried to explain that I was stressed and depressed because I felt sick all the time, not the other way around. In 1997, a new endocrinologist I was referred to at Columbia/Presbyterian was the first person to note that the severity of my osteoporosis and early onset was definitely not ‘‘normal,’’ and ordered more tests to determine an underlying cause. That year, I wore sunglasses to meetings when my eyes crusted and long sleeved shirts to cover my arms. In the summer, my friends thought I was being overly cautious about sun exposure, an excuse for which I was grateful. I did not mention that sunscreen burned the sores I was now constantly hiding. The dermatologist warned me about using too much cortisone cream, especially on my face, but it was the only thing that controlled the itching. At times, the itching would wake me up at night—I wanted to scratch my bones. By 1998, after trying cold compresses, yogurt, and anything else suggested, I used more and more topicals, took more nonsteroidal anti-inflammatory drugs, and became impossibly short tempered. My family bore the brunt of my distress. My husband, who had been saintly throughout all this, insisted I do ‘‘something, anything’’—see different doctors, take a break, find a way to ‘‘pull myself together.’’ I found it difficult to rationalize the gravity of the problem—people do not itch to death, so this needed to be put into perspective. I was told repeatedly by medical professionals that there was nothing wrong with me. So I stopped working and focused on rest, diet, and exercise. After three months of relaxed,
S30 Jones
healthy living, I felt worse than ever and hit a real physical and mental low. Luckily, the endocrinologist had sent a blood sample of mine to Peter Green, MD, at Columbia who was doing a random screening for celiac disease in osteoporosis patients and I was told that I had tested positive. Knowing little about the disease, I was scheduled for an endoscopy, which surprised me since I had no gastrointestinal symptoms. At the hospital, while Dr. Green explained the procedure, he saw me scratching my hands and face. Examining the sores, he asked if anyone had mentioned dermatitis herpetiformis to me? Would I have a biopsy? The endoscopy results were inconclusive—the mucosa was partly damaged, partly normal. The skin biopsy was not—I had dermatitis herpetiformis. The diagnosis dramatically changed my life. On a gluten-free diet, the joint pain and exhaustion started to fade within six to eight weeks. In three months, I felt better and had more energy than I had had in years. The clouds disappeared. Unfortunately, getting the DH under control was far more difficult. I found a dermatologist in New York who specialized in DH. She did not feel it necessary to put me on dapsone if I could control the sores through my diet. My skin continued to erupt, but within six months was dramatically better and the outbreaks were less severe and extensive. I saw her twice and seemed to be doing well on a strict gluten-free diet with topicals to control the occasional itching. I was again warned about cortisone use—but it was not easy living in my itchy skin when I had a flare-up. The dapsone alternative was not without its own dangers. While the outbreaks were much less severe, it was a roller-coaster ride to determine what was causing them. I was super-sensitive to accidental gluten ingestion, and found that iodine also seemed to flare up the DH. Nevertheless, my skin continued to improve. Every six months was better than the last.
J AM ACAD DERMATOL JULY 2004
Then, in mid-2001, I developed an almost constant red rash on parts of my face. My DH specialist was no longer in practice and I was referred to a dermatologist who diagnosed rosacea. She prescribed creams and scrubs in addition to the cortisone. When one area of my cheek started to bleed, I was referred to yet another dermatologist who took one look at me and said I was having a cortisone reaction and needed to stop using it on my face immediately. I was put on Protopic (tacrolimus) and went through a rough six weeks, but stopping the cortisone eventually eliminated the rash. The final chapter, or perhaps coda, to the story occurred in early 2003. I developed sores all over my arms and hands that looked and itched like DH, although they were not always mirrored. I was (and am) following a strict gluten-free diet, but I also began taking dapsone. A month later, I had a systemic allergic reaction to the drug. On re-examining my arms, the dermatologist noted that there are eczemas that are almost identical to DH—which is probably exactly what I had developed. Tacrolimus is now the cream of choice. I have not used the fluocinonide. I keep it for itching emergencies. The lack of knowledge about DH and the emphasis on treating symptoms rather than exploring causes made my journey to diagnosis a disturbing and painful one. I was given creams that covered up the problem and created others. Nevertheless, I consider the DH diagnosis one of the best things that has happened in my adult life—ironically, it took a gastroenterologist to see it. My symptoms finally had a name and one voice—I only wish it had spoken sooner. For more information, please visit the Celiac Disease Center at Columbia Web site at www. celiacdiseasecenter.columbia.edu or the Gluten Intolerance Group Web site at www.gluten.net.