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Scleroderma

Background

Scleroderma (hard skin) is a disease of the connective tissue, immune system and blood vessels with its onset being highest in the fourth and fifth decade of life and it is more common in women than men. Although it typically presents with tautness and discoloration of the affected skin, it may also affect the internal organs such as the heart, lungs, kidneys and gastrointestinal tract.

Credits

Medical text written October 2010 by Dr Voon H Ong, Consultant Rheumatologist, Royal Free Hospital, London, UK.

What are the symptoms?

Scleroderma (or Systemic Sclerosis also known as SSc) is broadly divided into Localised form meaning that it affects small areas of the skin, such as Morphoea or Linear Scleroderma and Systemic form, which affects multiple body systems.

The systemic form of SSc is further divided into two major groups based on extent of the skin affected.  Diffuse Cutaneous SSc has widespread skin involvement and the second type, Limited Cutaneous SSc affects the skin over the hands, lower arms, feet, lower legs and face. The limited type is approximately five times more common than the diffuse type.

In the diffuse type of SSc, internal organs may be affected early on in the illness. In the limited form, the major problems tend to occur later and the gut (bloating, diarrhoea and incontinence) and lung (usually Pulmonary Hypertension with raised blood pressure within the major vessels from the lung to the heart) may be affected. In addition, there may be calcinosis (deposits of calcium which mass under the skin and protrude), dry eyes and mouth, ulceration and Raynaud's disease (see entry Raynaud's Phenomenon).

Juvenile Scleroderma differs from the adult disease in that localised forms predominate. The juvenile disease attacks particularly the skin, muscles, joints, tendons and bones, with internal organ involvement a rarity. Childhood Morphoea may last for several years then resolve spontaneously, but the linear form may lead to growth defects, which require immediate treatment including physiotherapy.

What are the causes?

The precise causes for the illness are unclear but the scarring process that occurs in SSc is shared by other conditions such as Keloids (excessive skin scarring in response to injury) and Liver Cirrhosis.

How is it diagnosed?

The condition is diagnosed on the symptoms and clinical examination. The diagnosis may be supported by further investigations including blood tests for specific antibodies and where available, specialised imaging studies for abnormal nail-fold blood vessels formation with capillaroscopy may be helpful.

How is it treated?

Currently, the approach in treating the condition is aimed at the affected organs. For example, there are effective drugs to improve the circulation for Raynaud's disease. Topical treatments for skin include moisturisers to prevent dryness and cuts.

A multidisciplinary approach with multiple specialists including rheumatologist is required as SSc tends to involve several different organ systems. Children with SSc will be treated by either a paediatric dermatologist or rheumatologist together with multi disciplinary team. There are specific treatments available for Pulmonary Hypertension and it is therefore important that these complications are detected early on with regular investigations with Echocardiogram (EGC − a type heart scan) and lung function tests. Specific treatments for the kidney disease can be life saving. Other treatments are also available for the gut and lung problems.

Inheritance patterns and prenatal diagnosis

Is there support?

Scleroderma and Raynaud's UK (SRUK)

Helpline: 0800 311 2756
Email: info@sruk.co.uk
https://www.sruk.co.uk

SRUK are a Registered Charity in England and Wales No.1161828. They provide information and support to those affected by scleroderma or Raynaud's and they aim to raise awareness and understanding of these conditions.

Group details added October 2017.

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