Understanding Scleroderma

Scleroderma is a rare, chronic disease of the immune system, blood vessels and connective tissue. There are 2.5 million people worldwide who are living with scleroderma, and within the UK there are around 19,000 people diagnosed.

'Scleroderma' comes from the Greek, 'sclero,' meaning hard, and 'derma', meaning skin. It is an autoimmune condition, meaning that the immune system becomes overactive and attacks healthy tissue within the body. Hardening of the skin can be one of the first noticeable symptoms, as the body produces too much collagen. This excess of collagen can affect the skin, joints, tendons and internal organs. It causes scarring and stops the affected parts of the body from functioning normally.

The symptoms of scleroderma will vary for everyone, and the severity will depend upon how the disease affects each individual. No two cases will be the same.

Typical symptoms include hardening of the skin, swelling of the hands and feet, joint pain and stiffness and damage to the blood vessels, which leads to a physical over-reaction to the cold or emotional stress known as Raynauds disease'.

There are two main types of scleroderma: 'localised' and 'systemic':

Localised scleroderma

  • 'Morphea' is the name given to localised patches of hardened skin that appear smooth and shiny. They usually appear on the trunk, but they can affect any part of the body. The condition is painless and there are normally no other symptoms or additional problems.
  • 'Linear' scleroderma means that the skin is affected in the form of a line, usually along an arm or a leg. The skin appears shiny, discoloured or scarred, and often feels tight and uncomfortable. In children, this should be monitored carefully because the ongoing growth of the limbs can be affected
  • 'En coup de sabre' literally means 'cut of a sword'. This form of scleroderma presents on the scalp and temple in children. If the affected area is confined to the scalp then the problem is mainly cosmetic, although the underlying bone may be affected
  • 'Parry Romberg syndrome' also affects children and presents on the face, and occasionally the tongue. This requires careful monitoring as it may affect the growth of the facial bones 

Systemic sclerosis

In systemic sclerosis, the internal organs are affected as well as the skin, including the heart, oesophagus, blood vessels, kidneys, lungs, blood pressure and digestive system. There are two kinds of systemic sclerosis.

People with limited systemic sclerosis have often lived with Raynaud's syndrome for a long time. The condition progresses gradually, and usually only affects the face, the hands, the arms below the elbows, the feet and the legs below the knees - although the lungs and digestive system may be affected over time. Symptoms can include thickening of the skin, heartburn and problems with swallowing.

Diffuse systemic sclerosis is more likely to affect the whole body, and in some cases there can be potentially serious complications involving the heart, lungs and kidneys. Common symptoms include fatigue, joint pain and stiffness.

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