Conditions

Scleroderma: when the skin and organs harden

An autoimmune disease producing too much collagen

Dr. Camille Roux

Internal medicine physician

6 min read
Medical illustration of Scleroderma: when the skin and organs harden

In simple terms

Localised versus systemic forms, the early signs, and the treatments that changed the outlook.

What it is, in plain words

Scleroderma means hard skin. The immune system triggers fibroblasts to overproduce collagen, and small blood vessels narrow. In localised forms only patches of skin are affected. In systemic sclerosis, skin thickening is accompanied by damage to lungs, gut, kidneys and heart, which determines the prognosis.

Signs to look out for

  • Raynaud's phenomenon, usually the first symptom, sometimes years ahead
  • Puffy then tight, shiny skin of the fingers, later the face, limiting mouth opening
  • Heartburn, difficulty swallowing, bloating and alternating bowel habit
  • Breathlessness and dry cough from lung fibrosis or pulmonary hypertension
  • Small red spots on the face and hands, fingertip ulcers and calcium deposits

Why it happens

The cause is unknown; it is more common in women aged 30 to 50. Certain occupational exposures such as silica are associated with a higher risk.

How doctors confirm it

Specific antibodies help classify the disease and predict complications. Nailfold capillaroscopy, lung function tests with diffusion capacity, high-resolution chest CT, echocardiography and regular blood pressure and kidney monitoring form the standard assessment.

Treatments and solutions

  • Organ-directed treatment: proton pump inhibitors for reflux, calcium channel blockers and sildenafil for Raynaud's and ulcers
  • Immunosuppression with mycophenolate, or tocilizumab, and antifibrotic nintedanib for progressive lung fibrosis
  • ACE inhibitors urgently for scleroderma renal crisis, a treatment that transformed survival
  • Specific pulmonary hypertension drugs when it is detected on screening
  • Physiotherapy and hand exercises to preserve movement, plus stem cell transplant in selected rapidly progressive cases

Living with it day to day

Regular annual screening of lungs and heart is essential even when you feel well, because these complications are silent early and much more treatable when caught then.

When to seek medical help

Contact your specialist urgently for new high blood pressure with headache, a rapid rise in breathlessness, a non-healing fingertip ulcer, or difficulty swallowing solids.

This article explains general health information in simple language. It does not replace advice from your own doctor.

The words explained

Medical terms used above, in everyday language.

Symptom
A sign you can feel or see that something is wrong, like pain, tiredness or a rash.
Immune system
Your body's defence team: cells that fight germs and repair damage.
Blood pressure
The force of blood pushing against your artery walls. Too high, over years, damages the heart and vessels.
Screening
A check-up done before you feel ill, to catch a problem early.
Antibody
A protein your body makes to recognise and neutralise a specific germ.

When should you see a doctor?

  • Your symptoms last more than a few weeks or keep coming back.
  • They get worse quickly, or stop you doing everyday things.
  • You notice sudden pain, breathing trouble, fainting or bleeding — seek urgent care.
  • You already take medication and want to change anything about it.

This article explains general health information. It does not replace advice from your own doctor.

Frequently asked questions

What is Scleroderma: when the skin and organs harden?
Localised versus systemic forms, the early signs, and the treatments that changed the outlook.
What are the main symptoms or signs of Scleroderma: when the skin and organs harden?
Common signs vary from person to person. The article above lists the symptoms to watch for, how they progress, and when they need medical attention.
When should you see a doctor for Scleroderma: when the skin and organs harden?
See a doctor if symptoms last more than a few weeks, get worse quickly, stop you doing everyday things, or if you notice sudden pain, breathing trouble, fainting or bleeding.