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Skin itch caused by rare disorder

Dear Dr. Gott: Would you like one more letter about a severe-itching diagnosis? I was in my 60s when mine started. I did not have a rash or any visible cause for it, but it was horrible. I would get up at night and take a cold shower or rub ice all over my body. The scratching caused bleeding and sores, so I went to my dermatologist.

After my third visit, he recommended another dermatologist, who treated me for scabies. When nothing worked, she said she couldn't help me. It was awful: I couldn't sleep or eat.

Still another dermatologist was recommended. He tried a few things that didn't help, then decided to do a skin biopsy. So, lo and behold, we had a name for it: bullous pemphigoid. He put me on prednisone. I had a terrible reaction to that and lost all muscle control, and fell and broke my ankle in two places. The prednisone was then stopped, and he put me on Imuran and an antibiotic. That was the magic potion. My itch started clearing up, but it took a while. Now, 10 years later, I'm still taking the Imuran because I have spots that still break out from time to time. The dosage is gradually being reduced. I am so grateful to my doctor. I don't know why the other doctors didn't do a skin biopsy.

Reply: Bullous pemphigoid is a rare, chronic skin condition that causes fluid-filled blisters to form, usually on the arms, legs or trunk. The disorder is thought to be autoimmune but the exact cause is still unknown. It usually affects people over the age of 60.

Common symptoms include redness, rash, irritation, blisters, itching, hive-like lesions, bleeding gums and mouth sores. Mild cases may not develop into blisters. Instead, slight redness and irritation may occur. Severe cases may leak fluid, appear crusty, become painful and develop into open sores.

Treatment includes oral or topical corticosteroids, such as prednisone or hydrocortisone, which reduce inflammation; immunosuppressant drugs that calm the immune response and reduce inflammation; or corticosteroid-sparing agents that help reduce the necessary dosage of steroid or immunosuppressant medications. It is also important to protect the skin from the sun by using sunscreen whenever going outside (regardless of the season); take supplements, such as calcium and vitamin D if on long-term steroid treatment; avoid injuries because of skin fragility; and, if the mouth is affected, avoid crunchy foods, such as raw fruits and vegetables, that may cause a flare-up.

With treatment, the condition usually recedes within one to five years. Untreated, it may disappear spontaneously but can take as long as 10 years, and there is no guarantee it will resolve on its own. Skin infections are common complications of the disorder, and keeping the skin clean and dry is important. Treatment to reduce symptoms will also help, since scratching often results in open wounds or tears in the skin, which then allows bacteria to enter. I cannot say why your first two dermatologists failed to order a skin biopsy and am disappointed to hear that they both gave up so easily. I also applaud your current dermatologist for sticking with you to determine the cause of your itching. To provide related information, I am sending you a copy of my Health Report 'Dermatitis, Eczema and Psoriasis'. Other readers who would like a copy should send a self-addressed stamped number 10 envelope and a cheque or money order for $2 to Newsletter, PO Box 167, Wickliffe, Ohio 44092. Be sure to mention the title.

Dr. Peter Gott is a retired physician and the author of 'Dr. Gott's No Flour, No Sugar Diet' and 'Dr. Gott's No Flour, No Sugar Cookbook'. Contact him c/o United Media, 200 Madison Avenue, fourth floor, New York, New York 10016.