#Scleroderma

Scleroderma, also known as Systemic Sclerosis is a rare autoimmune disease characterized by the hardening and tightening of the skin and connective tissue. Autoimmune disease occurs when your immune system which normally protects from foreign substances attacks the healthy cells by mistake. Scleroderma is characterized by buildup of scar tissue in the skin and other organs. Individuals with scleroderma overproduce collagen (a fibrous protein that makes up connective tissue, such as the skin), leading to the fibrosis of the skin and the internal organs. The term arises from the Greek word “sclera” meaning hard and “derma” meaning skin. Scleroderma is characterized by skin thickening, blood vessel disease, and inflammation. It affects women more than men and can affect at any age. There are 2 types of scleroderma. The two types can be differentiated by the extent of skin involvement.

• Localized sclerosis
• Systemic Sclerosis

Localized sclerosis

It usually affects only the skin, although it can spread to the muscles, joints and bone. It is less serious and has no impact on the internal organs. There are 2 kinds of localized sclerosis.

• Morphea: Morphea causes multiple oval shaped lesions in the skin. It involves the skin but not the internal organs. It appears as whitish or darkened patches of skin. It often appears on chest, stomach and back.
• Linear: It is localized usually to a lower extremity. It appears as band-like thickening of skin on the arms or legs and often occurs in children.

Systemic Sclerosis

It affects the whole body. It can affect the skin, blood vessels, muscle, joints, lungs, kidney, and other organs. It occurs in two forms.

• Limited Cutaneous Systemic Sclerosis: This often develops slowly. In Limited Cutaneous Systemic Sclerosis Syndrome, fibrosis is limited to the hands, arms, and face. It can also affect your lungs, esophagus, and blood vessels. It was formerly known as CREST, and is an acronym for the common features of the conditions. It is characterized by:
  • C: Calcinosis is an abnormal accumulation of calcium salts under the skin. It appears as hard whitish area in the superficial skin.
  • R: Raynaud’s phenomenon refers to the spasm of blood vessels of the fingers, toes, ears and nose. It usually occurs due to the exposure to the cold environment. The hands and feet turn white and blue, in response to cold or anxiety.
  • E: Esophageal dysmotility- It refers to the contractions occurring in the esophagus due to the pathological disruption.
  • S: Sclerodactyly- It is the thickening and tightening of the skin on the fingers, caused by an excess of collagen deposit in the skin layer.
  • T: Telangiectasias- These are tiny red spots caused by the swelling of tiny blood vessels.
• Diffuse Cutaneous Systemic Sclerosis: It progress quickly. It involves thickening of the skin of extremities, face and trunk. It may affect any part of the body such as blood vessels, kidneys, lungs, heart or the gastrointestinal tract. It can sometimes become life threatening.

Cause

The exact cause of Scleroderma is not known. It is believed to be an autoimmune disease, in which collagen is deposited in high amount throughout the body. This excess collagen causes the skin to thicken and harden. The exact cause of abnormal collagen production is not known.

Symptoms

The sign and symptoms of scleroderma vary from person to person. It depends on the type and severity of the scleroderma and the organs affected. Since Scleroderma is a systemic disease, it can affect any part of the body. The Hardening of the skin is the most noticeable symptom as the body produces too much collagen. Scleroderma commonly affects esophagus leading to heartburn. Other symptoms involve swelling of hand and feet, joint pain, fatigue, loss of appetite, hair loss and unexplained weight loss.

Raynaud phenomenon is a frequent complaint. In Raynaud phenomenon, the finger and toes turn white or blue in response to the cold temperature or emotional distress. Finger color changes are caused by spasm and narrowing of blood vessels. Raynaud phenomenon can also occur in people who don’t have scleroderma.

Diagnosis

The sign and symptom of scleroderma is similar to many other diseases. To diagnose scleroderma, your doctor will examine your skin for signs of hardening and thickening. Your doctor will use a range of other medical tests including blood test for antibodies, urine test, x-rays, ECG and biopsy to reach a diagnosis.

Treatment

There is no cure for scleroderma but treatment can help reduce the effects of the disease. The type of treatment you get will depend on how deeply scleroderma goes.

Medications

• To decrease the symptoms of Raynaud’s phenomenon, Calcium Channel Blockers are recommended.
• Colchicine: To control the hardening of the skin.
• Blood pressure medicine such as Angiotensin Converting Enzyme (ACE) Inhibitor like lisinopril can be helpful for high blood pressure or kidney disease.
• Heartburn: If digestive tract is involved proton pump inhibitor like omeprazole may be appropriate.
• If lungs are affected, corticosteroids can be helpful.

Lifestyle changes

Some symptoms of scleroderma can be managed by lifestyle changes, such as-

• Quit smoking
• If you have Raynaud’s phenomenon, avoid exposure to cold temperature.
• Exercise regularly
• Localized scleroderma may be treated with moisturizing cream.

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