Also known as chronic sclerosis, scleroderma is a chronic hardening and tightening of the skin and connective tissues. “Sclero” meaning hard and “derma” meaning skin. Scleroderma is generally classified as one of the autoimmune rheumatic diseases and is not contagious, infectious, cancerous or malignant.
SIGNS AND SYMPTOMS
Signs and symptoms vary greatly because scleroderma varies from person to person. They also vary depending on the structures that are affected. Hardening of the skin is the one most visible manifestation. This disease affects only the skin in some people but in many others harms structures beyond the skin. The effects can range from very mild to life threatening. The seriousness depends on the parts of the body affected and the extent to which they are affected. A mild case can become serious if it is not treated properly. With prompt and proper diagnosis and treatment, symptoms can be minimized and the chance of irreversible damage will be lessened.
-Skin: nearly everyone experiences a hardening and tightening of patches of skin; the skin can appear shiny because it is so tight and movement of affected area may be restricted
-Fingers and Toes: one of the earliest signs of the disease is an exaggerated response to cold temperatures or emotional distress, which can cause numbness, pain or color changes in the fingers or toes (Raynaud’s phenomenon; can also occur in people who don’t have scleroderma)
-Digestive System: acid reflux is a problem; there is also problems with absorbing nutrients in the intestinal muscles that aren’t moving food properly through the intestines
-Heart: scarring increases the risk of abnormal heart beats (arrhythmias), congestive heart failure and pericarditis
-Lungs: scarring results in reduced lung function, reduced ability to breathe and reduced tolerance for exercise
-Kidneys: can develop elevated blood pressure and increased protein in the urine; more damage results in rapid renal failure
-Teeth: severe tightening of the facial skin can cause the mouth to become smaller and narrower which makes it difficult to brush or even have a professional cleaning; also, the person doesn’t produce normal amounts of saliva so there is an increased risk for decay
TYPES OF SCLERODERMA
–Localized Scleroderma: changes are only found in a few places on the skin or muscles and rarely spread elsewhere; generally mild; internal organs are usually not affected; people with localized rarely develop systemic scleroderma
–Morphea: a form of localized characterized by waxy patches on the skin of varying sizes, shapes and color; usually appears between the ages of 20 and 50, but often seen in young children
–Linear Scleroderma: a form of localized; frequently starts as a streak or line of hardened, waxy skin on an arm, leg or forehead; tends to involve deeper layers of skin as well as surface layers and sometimes affect the motion of joints which lie underneath; usually develops in childhood where the growth of involved limbs may be affected
–Systemic Scleroderma: may affect connective tissue in may parts of the body; can involve the skin, esophagus, GI Tract, lungs, kidneys, heart and other internal organs; can also affect the blood vessels, muscles and joints
Women are affected more than men. They outnumber men about 4 to 1. Factors other than gender, such as race and ethnic background may influence risk, age of onset and pattern of severity of internal organs involved ( the reasons for this are not clear).Choctaw Native Americans and African-Americans are more likely than those of American European descent to develop the type of scleroderma that affects internal organs.
The exact cause of scleroderma is still unknown but scientists and medical researchers are working hard to make those determinations. It is known that scleroderma results from an overproduction and accumulation of collagen. They also know that the body’s immune system plays a role. The immune system turns against the body, producing inflammation and the overproduction of collagen. Scleroderma is not directly inherited but some scientists feel that there is a slight predisposition to it in families with a history of rheumatic diseases.
Diagnosing scleroderma is difficult because it can take on so many forms and affect so many different areas of the body. There is a possibility of misdiagnosing or underdiagnosing cases because the symptoms are similar to other autoimmune diseases. Consultation with a rheumatologist (arthritis specialist) and a dermatologist (skin specialist) is important. A thorough physical examination is necessary. Blood tests are done to check for elevated blood levels of certain antibodies produced by the immune system. Doctors may perform a biopsy of the affected skin to be examined in the laboratory for abnormalities. Other possible tests include pulmonary function tests (breathing tests), CT scan of the lungs and an echocardiogram of the heart.
There is currently no cure for scleroderma. There is great variation in treatments since there is variation in the disease from one person to another. Treatment is directed at decreasing the activity of the immune system, helping control the symptoms or helping to prevent complications. Mild disease may not need medication at all and occasionally people can go off treatment when the disease is no longer active. Some cases of skin problems will fade away on their own in 3-5 years but the type that affects the internal organs ususally worsens with time. Often times, therapy, physical or occupational, is used to help manage pain, improve strength and mobility and help maintain independence with daily tasks. Surgery can be used as a last resort. Surgical options for complications include amputation and lung transplants. Lifestyle changes can also help when the person stays active, don’t smoke, manage heartburn and protects themselves from the cold.
Alternative medicine can also be a great option. Meditation and relaxation techniques can help one cope with frustrations and help relieve pain and fatigue. Professionals, such as therapists or behavior psychologists can help. Physical health can directly impact mental health. Denial, anger and frustration are common with chronic illness.
There are 20,000 to 200,000 U.S. cases of scleroderma a year. Scleroderma affects 300,000 Americans and (1/3 of those have the systemic form). Scleroderma can develop in every age group from infants to the elderly, but onset is most frequently between the ages of 25 to 55. Prognosis has improved dramatically over the past 30 years as the ability to detect and treat many of the problems of scleroderma has improved.
This blog post is for informational purposes only and is not intended to give any medical advice. If you think you may have scleroderma or have any questions or concerns, contact your own healthcare professionals.