Scleroderma: symptoms, early signs, what is condition that led to Scotland man having double hand transplant?

A man whose hands were left unusable due to scleroderma has undergone what is believed to be the world’s first double hand transplant for the condition.

Steven Gallagher, 48, from Dreghorn in North Ayrshire, was diagnosed with scleroderma after he developed an unusual rash on his cheeks and nose, and pains in his right arm.

But what is scleroderma?

Here’s what you need to know.

What is scleroderma?

Scleroderma is an uncommon condition that results in hard, thickened areas of skin and sometimes problems with internal organs and blood vessels.

There are several different types of scleroderma that can vary in severity, with some types being relatively mild and may eventually improve on their own, while others can lead to severe and life-threatening problems.

There two main types of scleroderma are:

• localised scleroderma – just affects the skin
• systemic sclerosis – may affect blood circulation and internal organs as well as the skin

What are the symptoms?

Localised scleroderma is the mildest form of the condition and often affects children, but can occur at any age.

This type just affects the skin, causing one or more hard patches to develop. Internal organs aren’t affected.

Exactly how the skin is affected depends on the type of localised scleroderma. There are two types, called morphoea and linear.

Morphoea:

• discoloured oval patches on the skin
• can appear anywhere on the body
• usually itchy
• patches may be hairless and shiny
• may improve after a few years and treatment may not be needed

Linear:

• thickened skin occurs in lines along the face, scalp, legs or arms
• occasionally affects underlying bone and muscle
• may improve after a few years, although can cause permanent growth problems, such as shortened limbs

However, in systemic sclerosis, internal organs can be affected as well as the skin. This type mostly affects women and usually develops between 30 and 50 years of age. Children are rarely affected.

There are two types of systemic sclerosis:

• limited cutaneous systemic sclerosis
• diffuse systemic sclerosis

Limited cutaneous systemic sclerosis:

• a milder form that only affects skin on the hands, lower arms, feet, lower legs and face, although it can eventually affect the lungs and digestive system too
• often starts as Raynaud’s (a circulation problem where fingers and toes turn white in the cold)
• other typical symptoms include thickening of the skin over the hands, feet and face, red spots on the skin, hard lumps under the skin, heartburn and problems swallowing (dysphagia)
• tends to get gradually worse over time, although it’s generally less severe than diffuse systemic sclerosis and can often be controlled with treatment

Diffuse systemic sclerosis:

• is more likely to affect internal organs
• skin changes can affect the whole body
• other symptoms can include weight loss, fatigue, and joint pain and stiffness
• symptoms come on suddenly and get worse quickly over the first few years, but then the condition normally settles and the skin may gradually improve

Organs such as the heart, lungs or kidneys can also be affected in some cases of systemic sclerosis. This can cause a range of potentially serious problems, including shortness of breath, high blood pressure and pulmonary hypertension (high blood pressure in the lungs).

What causes scleroderma?

The condition is caused by the immune system attacking the connective tissue under the skin and around internal organs and blood vessels, which then causes scarring and thickening of the tissue in these areas.

Is there a cure?

No, there’s not a cure for scleroderma, but most people with the condition can lead a full, productive life and the symptoms of the condition can usually be controlled by a range of different treatments.

How is scleroderma treated?

The NHS said the aim of treatment is to relieve symptoms, prevent the condition getting worse, detect and treat any complications, and help those with the condition maintain the use of affected parts of the body.

Common treatments include:

• medication to improve circulation
• medicines that reduce the activity of the immune system and slow the progression of the condition
• steroids to relieve joint and muscle problems
• moisturising affected areas of skin to help keep it supple and relieve itchiness
• various medicines to control other symptoms (such as pain, heartburn and high blood pressure)

Those with the condition will also need regular blood pressure checks and other tests to check for any problems with their organs.

If symptoms are severe, surgery may also be needed. For example, hard lumps under the skin may need to be removed and tightened muscles may need to be loosened.

There are also newer treatments that are currently being trialled, such as laser therapy and photodynamic therapy, which may improve the outcome of the condition for many people.