In short. Localised and systemic scleroderma compared: skin tightening, the organs at risk, and the monitoring that long-term disease requires.
Key points
- Morphea affects skin and the tissue below it, and does not shorten life.
- Systemic sclerosis can affect the lungs, heart, kidneys and gut.
- They are diagnosed and monitored differently, and only a careful assessment can tell them apart.
Scleroderma means hard skin: a group of conditions in which the skin, and sometimes the tissues beneath it, thicken. The word covers two very different situations. Localised scleroderma, usually called morphea, affects patches of skin and the tissue immediately beneath them and does not shorten life. Systemic sclerosis affects the skin more widely and can involve the lungs, heart, kidneys and digestive tract, and it is a serious disease. Telling the two apart is the first task of assessment.
Morphea
Morphea appears as one or more patches of firm, thickened skin, often with a violet or lilac border while active. It can be superficial or extend into fat, muscle and bone. A linear variant, more common in children, follows a line down a limb or across the face and can restrict growth of the limb or disturb development of the face and jaw. Morphea does not involve internal organs the way systemic sclerosis does, but it can cause permanent cosmetic and functional problems, especially across a joint or on the face.
Systemic sclerosis
Systemic sclerosis is divided into limited and diffuse forms. In limited disease, thickening is confined largely to the hands, forearms, feet and face, and colour change in the fingers in the cold often precedes it by years. In diffuse disease, thickening spreads rapidly to the trunk and the upper arms and legs, and internal organ involvement is more likely and more severe. Pulmonary fibrosis and pulmonary hypertension are the leading causes of death, which is why lung assessment sits at the centre of care.
Monitoring
Monitoring is organ-specific. Lung function tests and imaging detect interstitial lung disease, and other tests assess the heart and the lung circulation. Blood pressure is watched closely in early diffuse disease because of the risk of renal crisis, which is treatable when caught early. The digestive tract is assessed because reflux and difficulty swallowing are common and treatable. Each of these complications has a treatment that works better when it is started early.
Treatment classes
Treatment for morphea uses topical agents, ultraviolet phototherapy and, in active or deep disease, systemic immunosuppression including methotrexate, often with corticosteroids at the outset. Treatment for systemic sclerosis is directed at the complication in front of it: vasodilating agents for cold-induced colour change, agents for pulmonary fibrosis, drugs for pulmonary arterial hypertension, and drugs for gut symptoms. Immunosuppression is used for skin and lung disease in selected patients. Stem cell transplantation is reserved for a small group with severe, rapidly progressive disease.
Living with the change
Skin tightening alters the face and hands, restricts finger movement, and can make everyday tasks difficult. Cold protection, hand exercises, skin care and physiotherapy help to maintain function, and cold-induced colour change is managed by keeping warm and not smoking.
The distinction that matters most
Morphea affects skin and the tissue below it, and does not shorten life. Systemic sclerosis can affect the lungs, heart, kidneys and gut. They are diagnosed and monitored differently, and only a careful assessment can tell them apart.
Seek urgent care if
- Fingers or toes turn white and then blue, and stay painful or numb after rewarming.
- New breathlessness on exertion, or a persistent dry cough, in someone with scleroderma.
- Severe headache, visual disturbance, or a marked rise in blood pressure in early diffuse disease.
Frequently asked questions
How is morphea different from systemic sclerosis?
Morphea affects patches of skin and the tissue immediately beneath them and does not shorten life. Systemic sclerosis affects the skin more widely and can involve the lungs, heart, kidneys and digestive tract, and it is a serious disease. Telling the two apart is the first task of assessment.
Why is lung assessment central in systemic sclerosis?
Because pulmonary fibrosis and pulmonary hypertension are the leading causes of death. Lung function tests and imaging detect interstitial lung disease, and other tests assess the heart and the lung circulation. Each of these complications has a treatment that works better when it is started early.
Why is blood pressure watched so closely in early diffuse disease?
Because of the risk of renal crisis, a kidney complication that is treatable when caught early. A marked rise in blood pressure, a severe headache or visual disturbance in someone with early diffuse disease needs urgent care rather than a routine appointment.
Risks & Limitations
- These are chronic conditions that are managed rather than cured, and flares are expected along the way.
- Immunosuppressive treatment raises the risk of infection and needs regular blood monitoring.
- Long-term systemic corticosteroids carry real side effects, including bone, metabolic and eye complications.
- Steroid-sparing agents and biologics have their own risks, including blood count, liver and kidney effects.
- Treatment is usually long term, and relapse is common when it is reduced or stopped.
- No treatment works for everyone, response cannot be predicted, and nothing here is guaranteed.
- Some scarring, pigment change and hair loss may be permanent even with good control.
When to Seek Care
- Widespread blistering, or raw eroded skin that is spreading — seek urgent care.
- Blistering or erosions in the mouth or throat that make eating or drinking difficult — seek urgent care.
- Fever with a rash, especially while on immunosuppressive treatment.
- Sudden severe skin pain or peeling of the skin — seek emergency care immediately.
- New breathlessness or chest pain, which may indicate involvement beyond the skin — seek emergency care.
References & Further Reading
- American Academy of Dermatology (AAD) — public and clinical guidance
- International Pemphigus & Pemphigoid Foundation — patient and clinical resources
- Cochrane Library — systematic reviews in dermatology
- Chinese Medical Association, Dermatology Branch — clinical guidance
This page is patient education, not medical advice, and it does not cite individual studies. Discuss your own case with a qualified clinician. To arrange an assessment at Chongqing West District Hospital, see Contact & Consultation.