Scleroderma

Scleroderma (systemic sclerosis) is a rare autoimmune disease where the body's immune system causes abnormal hardening and tightening of the skin — and in systemic forms, damage to internal organs such as the lungs, heart, kidneys, and gut. Early diagnosis and specialist care can meaningfully change the outcome.

The two main types

• Localised scleroderma — patches (morphoea) or linear streaks of hardened skin; usually confined to the skin and milder overall.
• Systemic sclerosis — skin + internal organs. Divided into limited (CREST) and diffuse forms. More serious.

Common features

• Raynaud's phenomenon — fingers turn white, then blue, then red on cold or stress; often the earliest sign.
• Tight, shiny, thickened skin — usually starting on fingers (sclerodactyly).
• Small red spots on hands, lips (telangiectasia).
• Painful tips of fingers, sometimes ulcers or tissue loss.
• Heartburn, difficulty swallowing — oesophagus involvement.
• Shortness of breath, dry cough — lung involvement (interstitial lung disease or pulmonary hypertension).
• Sudden severe high blood pressure with kidney dysfunction — scleroderma renal crisis, a medical emergency.

Diagnosis

• Clinical examination plus autoantibodies (ANA, anti-centromere, anti-Scl-70, anti-RNA polymerase III).
• Nailfold capillaroscopy (a simple non-invasive test under a dermatologist/rheumatologist).
• Lung function tests, echocardiography (to screen for pulmonary hypertension), and sometimes HRCT chest.

Treatment — manage organ by organ

• No cure, but immunosuppressants can slow skin and lung disease.
• Anti-fibrotic class for scleroderma-associated interstitial lung disease — now available in India.
• ACE inhibitor at first sign of scleroderma renal crisis — life-saving.
• Calcium-channel blockers and lifestyle protection for Raynaud's.
• PPIs and dietary changes for reflux.
• Pulmonary hypertension treatments at specialist centres.
• Physiotherapy and hand exercises to preserve function.
• Mental-health and social support — patient groups exist in most metros (Indian Rheumatism Association and other disease-specific networks).

See a doctor

• New Raynaud's phenomenon with swollen fingers, joint pain, or tight skin.
• Progressive shortness of breath or dry cough.
• Severe heartburn or swallowing difficulty.
• Sudden severe rise in blood pressure with headache or breathlessness — renal crisis, emergency.