What is Scleroderma?
Scleroderma (also known as systemic sclerosis) is a rare, chronic autoimmune condition in which the immune system triggers an overproduction of collagen, causing skin and connective tissues to harden and tighten. The name comes from the Greek words for hard (sclero) and skin (derma). Scleroderma can range from a mild condition affecting only the skin to a severe systemic disease involving the lungs, kidneys, heart, and digestive system.
Types
- Limited cutaneous scleroderma (lcSSc) — skin thickening limited to the hands, face, and lower arms; associated with CREST syndrome (Calcinosis, Raynaud's, Oesophageal dysmotility, Sclerodactyly, Telangiectasia)
- Diffuse cutaneous scleroderma (dcSSc) — more widespread skin involvement and higher risk of organ involvement
- Localised scleroderma (morphoea) — affects only the skin, not internal organs
Symptoms
- Skin thickening, hardening, and tightening
- Raynaud's phenomenon — almost universal in scleroderma
- Puffy or swollen hands and fingers
- Difficulty swallowing (oesophageal involvement)
- Heartburn and reflux
- Joint pain and stiffness
- Lung fibrosis or pulmonary hypertension
- Telangiectasia — small red spots from dilated blood vessels
Treatment
There is no cure for scleroderma. Treatment focuses on managing specific symptoms and preventing organ damage. This may include medications for Raynaud's, immunosuppressants for skin and lung disease, proton pump inhibitors for reflux, and specialist management for pulmonary hypertension or kidney crisis.