A man whose hands were unusable due to scleroderma underwent what is believed to be the world’s first double hand transplant for the condition.
Steven Gallagher, 48, from Dreghorn in North Ayrshire, has been diagnosed with scleroderma after developing an unusual rash on his cheeks and nose and pain in his right arm.
Here’s what you need to know.
What is scleroderma?
Scleroderma is a rare condition that causes hard, thickened patches of skin and sometimes problems with internal organs and blood vessels.
There are different types of scleroderma that can vary in severity, with some types being relatively mild and eventually getting better on their own, while others can lead to serious and life-threatening problems.
There are two main types of scleroderma:
- localized scleroderma – affects only the skin
- systemic sclerosis – can affect blood flow and internal organs, as well as the skin
What are the symptoms?
Localized scleroderma is the mildest form of the disease and often affects children but can occur at any age.
This type only affects the skin, causing one or more hard patches to develop. Internal organs are not affected.
Exactly how the skin is affected depends on the type of localized scleroderma. There are two species, called morphoea and linear.
- discolored oval patches on the skin
- can appear anywhere on the body
- usually itchy
- Patches can be hairless and shiny
- may improve after a few years and treatment may not be necessary
- Thickened skin appears in lines along the face, scalp, legs, or arms
- occasionally affects underlying bones and muscles
- may improve after a few years but may cause permanent growth problems such as B. Shortened limbs
In systemic sclerosis, however, internal organs can also be affected in addition to the skin. This type mainly affects women and usually develops between the ages of 30 and 50. 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 affects only the skin on the hands, forearms, feet, lower legs, and face, although it can eventually affect the lungs and digestive system as well
- often begins as Raynaud’s (a circulatory problem where fingers and toes turn white in the cold)
- Other typical symptoms are thickening of the skin on the hands, feet and face, red patches on the skin, hard lumps under the skin, heartburn and difficulty swallowing (dysphagia)
- tends to gradually worsen over time, although it is generally less severe than diffuse systemic sclerosis and can often be controlled with treatment
Diffuse systemic sclerosis:
- tends to affect the 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 quickly worsen for the first few years, but then the condition usually settles down and the skin may gradually improve
In some cases, systemic sclerosis can also affect organs such as the heart, lungs or kidneys. This can cause a number 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 leads to scarring and thickening of the tissue in those areas.
Is there a cure?
No, there is no cure for scleroderma, but most people with the condition can live full, productive lives, and the symptoms of the condition can usually be controlled with a number of different treatments.
How is scleroderma treated?
The NHS said the aim of treatment is to relieve symptoms, prevent the condition from getting worse, identify and treat complications and help those affected maintain use of affected body parts.
Common treatments include:
- Drugs to improve blood circulation
- Medicines that reduce the activity of the immune system and slow down the progression of the disease
- Steroids to relieve joint and muscle problems
- Moisturizing affected areas of skin to keep it supple and relieve itching
- various medicines to control other symptoms (such as pain, heartburn and high blood pressure)
Those with the condition also need regular blood pressure checks and other tests to check for problems with their organs.
If symptoms are severe, surgery may also be needed. For example, hard knots under the skin must be removed and tense muscles relaxed.
There are also newer treatments currently being tried, such as laser therapy and photodynamic therapy, that may improve the outcome of the condition for many people.
https://www.nationalworld.com/health/scleroderma-symptoms-early-signs-what-is-condition-scotland-man-double-hand-transplant-3709813 What is scleroderma? Symptoms explained