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Systemic Sclerosis (Scleroderma)

Systemic Sclerosis (Scleroderma) - What it is

Scleroderma (also known as systemic sclerosis) literally means “hard skin”. The disorder is characterised by abnormalities of the blood vessels, connective tissue and the immune system. It can affect the skin, digestive system, heart, lungs, muscles and joints.

What causes scleroderma is currently unknown. Some unknown factor triggers an overproduction of collagen (a type of protein that is the main component of the connective tissue) causing thickening, hardening and scarring of the skin and organs throughout the body. 

What happens when I have scleroderma? 

In scleroderma, the body’s faulty immune system stimulates the fibroblast cells to produce excess collagen. How the body manages and ‘stores’ the excess collagen gives rise to the two major classifications of scleroderma: Limited Scleroderma and Diffuse Scleroderma with different patterns of involvement and symptoms. Let's take a closer look at each of them. 

1. Limited scleroderma 

In limited scleroderma, the skin involvement is less widespread and is typically confined to the fingers, hands, lower arms, lower legs and the face. Limited scleroderma is sometimes called CREST syndrome, which represents the initial letters of 5 common symptoms:
  • Calcinosis: Calcium deposits form under the skin around the elbows, knees or fingers, causing the affected regions of the skin to appear as hard, whitish areas. These deposits can feel tender and sometimes have a tendency to break through the skin, forming painful ulcers.
  • Raynaud’s phenomenon: Excess collagen deposits cause narrowing of small blood vessels and decreased blood flow to the extremities, such as the fingers and toes, which often turn white/blue in response to cold temperatures or emotional stress. In extreme cases, this may lead to finger ulcers. Around 90% of patients with limited and diffuse scleroderma will experience this.
  • Esophageal dysfunction: When scleroderma involves the muscles of the digestive system, some patients may develop an abnormally wide esophagus, experiencing heartburn, acid reflux, or swallowing difficulties.
  • Sclerodactyly: Excess collagen is deposited as areas of thick and shiny skin on the fingers and sometimes the toes. The layer of hard skin often makes it difficult to bend or straighten the fingers.
  • Telangiectasia: Small clusters of dilated blood vessels that appear as red spots may develop on the hands and face. These spots are not usually painful, but can cause cosmetic problems.
Limited scleroderma commonly manifests itself slowly over a period of 10 to 20 years. Raynaud’s phenomenon often precedes the skin involvement by many years, followed by other areas of involvement. One of the late manifestations may include heart/ lung involvement called pulmonary arterial hypertension (increased blood pressure in the artery that carries blood from the heart to the lungs).

2. Diffuse scleroderma 

Skin involvement in diffuse scleroderma is more widespread and has a much faster onset and progression than limited scleroderma. More importantly, people with diffuse scleroderma have a higher risk of developing hardening of the internal organs such as the lungs, kidneys and heart.

Am I at risk of scleroderma?

Scleroderma is not a common condition. An estimated 20 to 200 people per million are affected worldwide, with the age of onset usually between 30 to 50 years. Women are 4 times more likely to develop the condition than men.

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