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Scleroderma: The Slowly Contracting Skin of Steel
Pathology Perspectives

By Kalyani Premkumar

Originally published in Massage Bodywork magazine, August/September 2005. Copyright 2005. Associated Bodywork and Massage Professionals. All rights reserved.

The first documented account of scleroderma was in 1754 by Dr. Carlo Curzio of Naples, Italy, who described his 17-year-old patient with the following condition: "Her complaint was an excessive tension and hardness of her skin over all her body, by which she found herself so bound and straightened she could hardly move her limbs. Nor could she fully close her eyelids or open her mouth, due to the firmness of the skin and membrane."

In Greek, the word scleroderma means "hard skin" -- a common feature of the disease. But scleroderma does not affect the skin alone. It is a complex, chronic, degenerative, autoimmune disorder that can produce changes in many other parts of the body, including the digestive, respiratory, renal, musculoskeletal, and cardiovascular systems.


Commonality and Causes
Surprisingly, scleroderma is as common as multiple sclerosis and muscular dystrophy. It affects approximately 300,000 Americans every year, and more than 10,000 people with the serious form of this condition die annually. It affects more women than men (4-to-1) and, typically, people between the ages of 30 and 50 are more at risk.

The cause of this disorder is still not known. There is a genetic predisposition to the condition with a two-fold likelihood of a relative being diagnosed. However, there is also an environmental linkage to the etiology of the disease. Exposure to silica dust, certain drugs, organic solvents, and viral infections may serve as triggers to those who are predisposed to the condition. Workers in coal and gold mines are more at risk. Some have theorized it may be due to an immune reaction to fetal cells crossing the placenta during pregnancy. While the cause is still unknown, the disease process has been identified.

It is now accepted that there is vascular injury with inflammation in the target tissue. Fibroblasts are stimulated to produce more collagen than normal. However, even after the inflammation subsides, the scarring persists with reduced blood flow to the tissue in question. Blood vessels in the skin, lungs, kidneys, and gastrointestinal tract may be affected. In the skin, there may be thickening and scarring with reduced wound healing and ulcer formation. The skin becomes shiny and taut, eventually restricting movement (see Figures 1A and 1B, page 106). Raynaud's phenomenon (a condition where blood vessels go into a spasm when exposed to cold) is common in these individuals. Scarring in the lungs can lead to pulmonary hypertension and reduced blood flow to the kidneys can lead to an increase in blood pressure and kidney failure. In the gastrointestinal tract, scarring may result in narrowing of the intestines and gastroesophageal reflux.


Types of Scleroderma
Scleroderma has been broadly classified as localized and systemic. Each of these classifications has many subtypes. Localized scleroderma affects the skin and perhaps the underlying muscle and bone. It does not affect internal organs. Though the prognosis is better, skin changes can cause disfigurement. Some subtypes of localized scleroderma result in patches of skin changes, while others may have large dark patches of thick and tightened skin extending across the trunk, arms, or legs.

Systemic scleroderma, also known as systemic sclerosis, is a severe form that affects multiple organs. It can not only affect the skin, but also underlying blood vessels, muscles, and joints, as well as the gastrointestinal tract, kidneys, lungs, and heart.


What are the Symptoms?
Scleroderma may present in many different ways. In the skin, depending on the site, there may be ulcerations, tightness, swelling, itching, and difficulty in moving regions where fibrosis has occurred. There may be hypopigmentation or hyperpigmentation. Calcium deposits may be found under the skin and dryness of mucus membrane is a common symptom.

If the blood flow is affected, the skin may appear pale or blue. If the joints and muscles are affected, there may be difficulty in movement, swel-ling, and stiffness. Prolonged scarring may lead to contractures. If the gastrointestinal tract is affected, it may present as difficulty in swallowing, gastroesophageal reflux, nausea, vomiting, and weight loss. Kidney disease may lead to hypertension while lung changes result in pulmonary hypertension and difficulty breathing. Fibrosis in and around nerves may result in loss of sensation and motor function.

The course of the disease is variable. In some, symptoms become worse. In others, it may decrease or go away altogether. Unfortun-ately, there is no cure for the disease as yet.

Since scleroderma may affect multiple systems, many patients have the frustrating experience of being shuttled from one physician to another before an actual diagnosis is made. Scleroderma is best treated by specialists (i.e., rheumatologists who know most about the condition).


How is it Treated?
Currently, treatment is limited to containing the symptoms. With further research and discovery of the root cause of the problem, a cure may be found.

Vasodilators in various forms are given to treat Raynaud's syndrome. Anti-inflammatory drugs, corticosteroids, or immunosuppressants may be given to reduce fibroblastic activity and immunity and thereby inflammation and fibrosis. Drugs that suppress acid production may be given to those with gastroesophageal reflux. In order to prevent changes in blood pressure due to kidney or lung disease, drugs such as calcium channel blockers may be used to dilate blood vessels. Skin ointments may be prescribed to reduce dryness. And physiotherapy may be used to strengthen muscles and increase the range of motion. In order to minimize complications, it is important for the client to have a regular checkup so that symptoms of organ disease may be recognized before they manifest.


Role of Bodyworkers
Since the treatment given is symptomatic, bodyworkers can be of tremendous help to these patients. The therapist-client relationship is of great importance.

Scleroderma presents in a variety of ways, and therapy has to be tailored based on the symptoms and drug treatment regimen. A thorough history has to be taken and recorded. If the client has a history of gastrointestinal reflux, it may be wise to position her with the upper body raised. Use of moisturizing oil helps to reduce dryness. Due to reduced blood flow, the skin may be frail and cracked, and strokes must be adjusted accordingly. The therapist should be on the lookout for atrophy and ulceration of the skin. In those with contractures, range of motion may be restricted and movement should not be forced.

It is important for the therapist to be aware of the side effects of drugs that are being administered. For example, if on steroids, immunity may be suppressed. Some of the drugs given for hypertension may make the client prone to postural hypotension. Often, clients are on painkillers that may reduce their ability to give adequate feedback.


Further Research
There are still a number of key questions that need to be answered: How are the immune system, blood vessels, and fibroblasts related? What is responsible for triggering the process? At which stage can one intervene to cure or stop the progress of the disease?

With the establishment of research centers and support groups, progress is being made in terms of identifying better drugs for treatment and a cure for the disease. National registries have been established to track families and patients with scleroderma. DNA of patients is being studied to identify abnormal genes or gene expressions that are common across these patients. By identifying the proteins formed/not formed, drugs may be used to target these proteins. For example, specific receptors on the cell membrane of fibroblasts may be targeted to reduce collagen formation. As scleroderma is an autoimmune disease, the types of antibodies present in these patients is another area of research. The scleroderma research foundation, founded by Sharon Monsky, a scleroderma patient herself, is now leading the way in increasing public awareness, encouraging cutting-edge research to find a cure, providing information that may help scleroderma patients to better understand their disorder and more effectively manage its signs and symptoms.


Kalyani Premkumar, MBBS, M.D., MSc., Ph.D., a physician and certified massage therapist, is an assistant professor, Educational Support and Development, Faculty of Medicine, University of Saskatchewan; adjunct assistant professor, Department of Anatomy and Cell Biology, Faculty of Medicine, University of Calgary; tutor, Athabasca University; and an instructor at the Center for Complementary Health Education, Mount Royal College, Calgary, Canada. She is the author of The Massage Connection -- Anatomy and Physiology; Pathology A to Z -- A Handbook for Massage Therapists; and Medical Terminology -- a Beginner's Guide.


References
1 Scleroderma research foundation. Available at www.sclerodermaresearch.org.
2 UCSF Scleroderma Registry Clinic. Available at www.dermatology.ucsf.edu/autoimmune.
3 International Scleroderma Network. Available at www.sclero.org.
4 Cyberrounds rheumatology. Available at www.cyberounds.com/conf/rheumatology/2004-08-20.
5 Gilliland B. Systemic sclerosis (scleroderma) and related disorders. In: Kasper DL et al. editors. Harrison's Principles of Internal Medicine 16th Ed. New York: McGraw-Hill; 2005. Database title: STAT!Ref Online Electronic Medical Library. http://80-gateway.ut.ovid.com.ezproxy.lib.ucalgary.ca:2048/gw1/ovidweb.cgi. Accessed: 5/20/05.
6 Premkumar, K. Pathology A to Z -- A Handbook for Massage Therapists. 2nd Ed. Calgary: VanPub Books; 1999.





Resources
Scleroderma Foundation
12 Kent Way, Suite 101
Byfield, MA 01922
800/722-4673 or www.scleroderma.org

Scleroderma Research Foundation
220 Montgomery Street, Suite 1411
San Francisco, CA 94104
800/441-2873 or www.srfcure.org

International Scleroderma Network
7455 France Ave. S #266
Edina, MN 55435
800/564-7099 or www.sclero.org






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