Quick answer. Scleroderma is an autoimmune condition in which the body makes too much collagen, causing the skin – and in some people internal organs – to become hardened and tight. It ranges from milder forms affecting only the skin to systemic sclerosis, which can involve the gut, lungs, kidneys and heart, so specialist care and monitoring for organ involvement are important.
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A useful early clue: Raynaud’s phenomenon – fingers going white, then blue, then red and painful in the cold – is very often the first sign. New Raynaud’s together with skin changes deserves a medical review.
What scleroderma is
Scleroderma is a chronic autoimmune condition in which the immune system triggers overproduction of collagen, the protein that gives skin and connective tissue their structure. Too much collagen makes tissue thick, hard and tight. In the skin this is felt as tightening and stiffening; when internal organs are involved, the same scarring-type process can affect how they work. Scleroderma also involves small blood vessels, which become overreactive and narrowed – which is why circulation problems such as Raynaud’s are so closely linked. It is not contagious and not caused by anything the person did. (The name comes from the Greek “sclero” = hard and “derma” = skin.)
The two broad types
- Localised scleroderma affects mainly the skin and usually does not involve internal organs. Its forms are morphea (oval patches of hardened, discoloured skin) and linear scleroderma (a line or streak of thickened skin, often on a limb or the face);
- Systemic sclerosis can affect the skin and internal organs. Within it there are two subtypes: limited cutaneous (skin thickening confined mainly to the hands, forearms, lower legs and face; tends to develop more slowly) and diffuse cutaneous (more widespread skin involvement including the trunk; tends to come on faster and carries a higher risk of early internal-organ involvement).
Knowing the type helps the specialist decide how closely to monitor for organ problems.
Who gets it
Scleroderma is more common in women than men and usually develops in adults (the precise peak-age range varies between sources, so treat any single figure as approximate). As with other autoimmune diseases, the exact cause is not fully understood and is thought to involve a genetic predisposition together with environmental factors. It is not directly inherited in a simple way, and it is not contagious.
Symptoms – skin and circulation
Raynaud’s phenomenon is very often the earliest sign: in cold or with stress, the fingers (and sometimes toes) go white, then blue, then red as blood flow returns, often with numbness, tingling or pain. Raynaud’s is common in the general population and mostly harmless on its own, but new Raynaud’s together with skin changes should prompt review. Skin thickening and tightening typically starts in the fingers and hands (sclerodactyly), and the skin can feel shiny, tight and hard to pinch; many people first notice puffy or swollen hands, especially in the morning. Over time, skin colour changes, tight facial skin, small dilated blood vessels on the skin and calcium deposits under the skin can occur.
Possible internal organ involvement
In systemic sclerosis the same process can affect organs, which is why monitoring matters:
- Gut – the most commonly affected internal system: acid reflux/GORD and swallowing difficulties (the oesophagus becoming less mobile), and further down, bloating, constipation, diarrhoea or absorption problems;
- Lungs – interstitial lung disease (scarring, causing breathlessness and cough) and pulmonary hypertension (raised pressure in the lung arteries, which is serious) – key reasons for regular lung screening;
- Kidneys – scleroderma renal crisis, an uncommon but serious sudden rise in blood pressure with kidney impairment, needing urgent treatment;
- Heart and joints – the heart muscle and electrical system can be affected in some people, and joints can be painful and stiff, partly from tight overlying skin.
How it is diagnosed
Diagnosis brings together clinical features (the pattern of Raynaud’s, skin thickening and any organ symptoms), blood tests / autoantibodies (certain autoantibodies are strongly associated and help predict organ risk, interpreted by the specialist), nailfold capillaroscopy (a simple, painless look at the tiny blood vessels at the base of the fingernails), and organ screening – because internal involvement can be silent early on, baseline and periodic lung function tests, chest imaging and an echocardiogram (which also screens for pulmonary hypertension), plus blood-pressure and kidney monitoring.
Treatment and specialist care
There is no cure, but a great deal can be done to manage symptoms and protect organs, tailored to what’s affected: warmth and blood-flow measures (and stopping smoking) for Raynaud’s; acid-reducing medicines for reflux; monitoring and specific treatment for lung and kidney involvement; immunosuppression in some people, particularly with more active or diffuse disease; and physiotherapy and hand therapy to keep skin and joints supple. Care is best led by a rheumatologist, often with a team, with regular organ monitoring built in. The outlook varies widely by type and organ involvement – many people with limited disease do well over the long term – so the message is hopeful but honest. See a doctor if you develop new Raynaud’s, especially with skin tightening or puffy hands, or systemic symptoms such as unexplained breathlessness or persistent reflux.
Related autoimmune & rheumatology topics
- Autoimmune disease (overview)
- Lupus nephritis
- Antiphospholipid syndrome
- Polymyositis & dermatomyositis
- Juvenile idiopathic arthritis
- Pseudogout (CPPD)
- Joint hypermobility
- Autoimmune hepatitis
- Behcet’s disease
- Lupus (SLE)
- Rheumatoid arthritis
- Rheumatologists in Singapore
Sources
- Scleroderma & Raynaud’s UK (SRUK) — scleroderma types (localised vs systemic), Raynaud’s, living with the condition.
- NHS — Scleroderma: symptoms, types and treatment.
- Cleveland Clinic — limited vs diffuse systemic sclerosis, collagen overproduction and organ involvement.
- Mayo Clinic / NHLBI — diagnosis, organ complications (ILD, pulmonary hypertension, renal crisis) and treatment.
