Scleroderma

There are two main types of scleroderma:

• localised scleroderma, which just affects the skin
• systemic sclerosis, which affects the internal organs as well as the skin

Localised scleroderma – Thisis the mildest form of the disease and can occur at any age. The disease just affects the skin, causing one or more hard patches. The internal organs are not affected.

Exactly how the skin is affected depends on the type of localised scleroderma. There are two types, which are summarised as follows:

Morphoea:

• oval skin patches, which are usually itchy
• often only a few patches
• can affect skin anywhere on the body
• patches may be lighter or darker than normal skin and hairless
• patches may fade after a number of years
• treatment may not be needed

Linear:

• thickened skin occurs in lines (like a knife wound) across the face or scalp, leg or arm
• children commonly have a thickened line of skin down their leg
• lines persist for longer than morphoea patches
• also affects underlying bone and muscle
• can lead to growth problems in children
• may cause deformity

Systemic sclerosis – This affects the organs as well as the skin. Most people with systemic sclerosis have problems with their gut, such as heartburn and swallowing problems.

The skin becomes puffy and thickens, which can restrict joint movement. The fingers and toes may start to swell like sausages, before the skin becomes hard and tight. The facial skin also becomes tight, especially around the mouth.

Other general symptoms may include:

• hair loss
• weight loss
• fatigue
• joint pain and stiffness
• red spots (broken blood vessels) on the face, hands and arms

Systemic sclerosis most commonly occurs in adults aged between 25 and 55. Children are rarely affected.

There are two types of systemic sclerosis. These are:

Limited Systemic Sclerosis:

• a milder form of systemic sclerosis, which progresses slowly
• often starts as Raynaud’s disease (a circulation problem where fingers and toes turn white in the cold)
• other typical symptoms are thickening of the skin over the extremities and face, red spots (dilated blood vessels) on the skin, and hard lumps of calcium underneath the skin (especially the fingertips)

Diffuse Systemic Sclerosis:

• skin changes can affect the whole body
• symptoms come on suddenly and get worse quickly over the first few years, and then the disease settles and the skin may improve

In some cases of systemic sclerosis the heart, lungs or kidneys are affected. This can cause a range of symptoms such as shortness of breath, high blood pressure and pulmonary hypertension (high blood pressure in the lungs).

If the bowel is affected, it can lead to diarrhoea, constipation or faecal incontinence (leaking of stools).