Scleroderma :- Types, Symptoms, Cause, Risk Factor, Diagnosis, Treatment, Prevention and General Home Care of Scleroderma

Derived from the Greek words "sklerosis," meaning hardness, and "derma," meaning skin, scleroderma literally means hard skin. Though it is often referred to as if it were a single disease, scleroderma is really a symptom of a group of diseases that involve the abnormal growth of connective tissue, which supports the skin and internal organs. It is sometimes used, therefore, as an umbrella term for these disorders. In some forms of scleroderma, hard, tight skin is the extent of this abnormal process. In other forms, however, the problem goes much deeper, affecting blood vessels and internal organs, such as the heart, lungs, and kidneys.

Scleroderma is one of a group of arthritic conditions called connective tissue disorders. The term rheumatic disease refers to a group of conditions characterized by inflammation and/or pain in the muscles, joints, or fibrous tissue. A connective tissue disease is one that affects the major substances in the skin, tendons, and bones. In these disorders, a person's antibodies are directed against his or her own tissues.

Types of Scleroderma

Scleroderma can be classified in terms of the degree and location of the skin involvement. Accordingly, scleroderma has been categorized into two major groups, diffuse and limited.

There are two basic types of scleroderma:

•  Localized

•  Generalized.

In most cases, localized scleroderma is a more mild form of the disease. It is characterized by thickening or hardening of the skin on the arms, legs, and face. Localized scleroderma usually does not affect the trunk (chest, back, and abdomen).

There are two forms of localized

1. Morphea, which causes hard, oval-shaped patches on the skin

2.linear, which causes lines or streaks of hardened skin on the arms, legs, and forehead.

Localized scleroderma features CREST syndrome, which involves the following:

C-Calcinosis (abnormal calcium deposits in the skin)

R-Raynaud's syndrome (reduced blood flow to the extremities causing numbness, tingling, and burning; also called Raynaud's phenomenon)

E-Esophageal dysfunction

Sclerodactyly (hardening of the skin of the fingers and toes)

T-Telangiectasia (lesion caused by dilation of a group of blood vessels)

Generalized scleroderma may affect many parts of the body. It often involves thickening of the skin of the arms, legs, and trunk. There are two basic types of generalized scleroderma namely:

•  Limited

•  Diffuse.

Limited scleroderma usually develops gradually over time. It affects the skin first and then affects organs (e.g., gastrointestinal tract, heart, lungs, kidneys).

Diffuse scleroderma develops rapidly and causes thickening of the skin throughout the body. This type often affects internal organs.

Scleroderm Symptom

The disease may produce local or systemic symptoms. The course and severity of the disease varies widely in those affected.

Symptoms of scleroderma include;

Arthritis

Joint or bone aching

Weight loss

Fatigue

Raynaud's phenomenon

Stiffness of hands and feet

Discoloration of skin

Shortness of breath

Heartburn

Swallowing difficulties

Unexplained swelling, puffiness

Skin thickening, tightness

Dry mucus membranes

Skin is hard

Tight and mask-like facial skin

Ulcerations on fingertips or toes

Diarrhea

Constipation

Wrist pain

Wheezing

Hair loss

Scleroderma Cause

The cause of scleroderma is unknown. Excess collagen deposits in the skin and other organs produce the symptoms. Damage to small blood vessels within the skin and affected organs also occurs. In the skin, ulceration, calcification, and changes in pigmentation may occur.

Systemic features may include fibrosis and degeneration of the heart, lungs, kidneys and gastrointestinal tract.

A combination of factors (e.g., genetic, environmental) may contribute to development of the disease. Certain inherited antigens (cell markers), called human leukocyte antigens (HLA) are associated with an increased risk for scleroderma. Scientists are certain that people cannot catch it from or transmit it to others. Scientists suspect that scleroderma comes from several factors that may include:

Abnormal immune or inflammatory activity: Like many other rheumatic disorders, scleroderma is believed to be an autoimmune disease. An autoimmune disease is one in which the immune system, for unknown reasons, turns against one's own body. In scleroderma, the immune system is thought to stimulate cells called fibroblasts to produce too much collagen. In scleroderma, collagen forms thick connective tissue that builds up around the cells of the skin and internal organs. In milder forms, the effects of this buildup are limited to the skin and blood vessels. In more serious forms, it also can interfere with normal functioning of skin, blood vessels, joints, and internal organs.

Environmental factors : Research suggests that exposure to some environmental factors may trigger the disease in people who are genetically predisposed to it. Environmental risk factors include viral infections, certain adhesive and coating materials, exposure to silica dust (e.g., in coal and gold miners), exposure to certain chemicals (e.g., vinyl chloride, epoxy resin, pesticides) and solvents (e.g., benzene, toluene), and certain medications

Hormones: By the middle to late childbearing years (ages 30 to 55), women develop scleroderma at a rate 7 to 12 times higher than men. Because of female predominance at this and all ages, scientists suspect that something distinctly feminine, such as the hormone estrogen, plays a role in the disease. So far, the role of estrogen or other female hormones has not been proven.

Risk Factor of Scleroderma

Age: more common between the ages of 30 and 50

Gender: more common in women

Disease: diabetes.

Pregnancy: some research suggests that having children may increase a woman's risk of scleroderma. Scientists have learned that when a woman is pregnant, cells from her baby can pass through the placenta, enter her blood stream, and linger in her body--in some cases, for many years after the child's birth.

Scleroderma Diagnosis

If your doctor thinks you have scleroderma, he or she will usually refer you to a rheumatologist .A rheumatologist is a doctor who has received special training in the diagnosis and treatment of problems involving inflammation of the joints, muscles and other parts of the body. The doctor will ask about your symptoms and medical history, and perform a physical exam. There is not just one single symptom, sign or test that will give a diagnosis of scleroderma. Diagnosis is based on changes in the skin. Other tests may include:

Imaging tests - plain x-rays, computed tomography (CT) scan or magnetic resonance imaging (MRI) scan to look at internal organs

Blood tests.

Skin biopsy: If skin changes are already present the diagnosis may be simple and routine. In other cases where scleroderma is suspected, your doctor may wish to do a skin biopsy, which involves removing a small piece of skin to be analyzed in a laboratory.

Scleroderma Treatment

There is no cure for systemic sclerosis. The goals of treatment is to relieve symptoms and maintain function. Most treatments are used for a particular symptom or the particular organ involved. Treatment usually includes a combination of physical therapy and skin and joint protection techniques

Psoralen and ultraviolet A (PUVA) therapy may be used. This treatment involves exposing the patient to increasing amounts of ultraviolet A (UVA) rays with an oral or topical substance called psoralen, which makes the skin more sensitive to treatment and improves its effectiveness.

If sores (ulcers) develop on the skin, antibiotic ointments may be useful.

Joint and muscle pain and stiffness may be treated using nonsteroidal anti-inflammatories and, in severe cases narcotic pain relievers. Angiotensin-converting enzyme (ACE) inhibitors may be used to treat kidney involvement. In severe cases, dialysis or kidney (renal) transplant may be necessary.

Treatment for lung involvement depends on the severity of the condition. Calcium channel blockers, prostaglandins, antibiotics, high doses of corticosteroids may be prescribed

Raynaud's syndrome can be treated by avoiding stress and cold temperatures (dressing in layers) and using medications such as calcium channel blockers and vasodilating drugs, which increase blood flow.

Doctors often treat localized scleroderma with therapies such as moisturizers or corticosteroid medications that you apply to your skin. Corticosteroid medications impede your body's ability to make substances that can cause inflammation.

If the condition involves a lot of body area or an arm or a leg, your doctor may prescribe systemic drugs to try to halt the progression of the disease. Treatment of systemic scleroderma may be with drugs that improve blood flow, promote esophagus and bowel function, preserve kidney function and control high blood pressure.

Scleroderma Prevention

If you have scleroderma you should do things to care for your skin.

Learn as much as you can about the disease. Speak with people who are specialists in arthritis care can. They will provide you with the information you need.

Relaxing can help to relieve pain and stress. There are many ways to relax. Try deep breathing exercises. Listen to music or relaxation tapes. Meditate or pray.

Stay active. Exercise keeps your body flexible, improves circulation and relieves stiffness. Range-of-motion exercises can help keep your skin and joints flexible.

Protect yourself from the cold. In the winter use a humidifier in your home.

Keep your hands and feet warm. Wear gloves and warm socks in cold weather when your hands will be subjected to cold temperatures When you're outside in the cold, be sure to cover your face and head and wear layers of warm clothing.

Use creams and lotions to keep your skin from drying out. Lanolin cream, light mineral oil, petroleum jelly, cocoa butter lotion or baby oil work well at keeping your skin moist.

Avoid using strong soaps that contain perfumes or chemicals.

Put baby oil into your bath water.

Scleroderma General Home Care

Avoid smoking. Smoking causes blood vessels to shrink and will make the symptoms worse.

If you have trouble swallowing, be sure to chew your food slowly and many times. Eating smaller meals more frequently to help keep too much acid coming up from your stomach.

Drink fluids with your meal if your mouth is dry.

If your eyes are dry you can get artificial tears to use as drops in your eyes. There are also mouthwashes and sprays you can use if your mouth is very dry.

Try to protect areas of skin calcification from pressure and abrasion. In these hardened areas the skin can more easily break down and get infected. Keep areas of skin that may have opened very clean.

Raising the head of your bed, and not lying down right after meals can help you minimize acid coming back up into your esophagus.

Be kind to your body. After doing heavy work, or doing the same task over and over, stop. Slow down by doing an easy task, or by taking a rest.

Use your back, arms and legs in safe ways to avoid putting stress on joints

Use helpful devices such as a cart to carry your grocery bags, or an enlarged handle that fits over a knife handle so you can hold it easily. A cart will help you to walk more safely.

Scleroderma Complication

High blood pressure

Kidney failure

Lung problems

Malnutrition

Death may result from gastrointestinal, heart, kidney, or lung involvement

CALL THE DOCTOR NOW

Call the doctor if:

You're experiencing the early signs and symptoms of scleroderma such as:

Numbness

Pain or color changes in the skin of your extremities

Gradual hardening and tightening of your skin

Stiffness or pain in your joints.