Scleroderma

Also called Systemic sclerosis, Morphoea

Hardening of the skin, and sometimes of internal organs. Cold white fingers years earlier are usually the first sign anybody had.

What it is

An autoimmune condition causing excess collagen, which thickens and hardens tissue. Localised scleroderma, called morphoea, affects patches of skin only. Systemic sclerosis affects skin plus internal organs and is the serious form.

Almost everyone with systemic sclerosis has Raynaud's phenomenon first, often for years. Raynaud's alone is common and usually harmless; Raynaud's with skin thickening, ulcers on the fingertips, or abnormal nailfold capillaries is different and needs rheumatology assessment.

Two complications drive the outlook and both can be silent until advanced: pulmonary hypertension and lung fibrosis. Annual screening with lung function tests and an echocardiogram is standard care and is the single most important thing to ask for.

A scleroderma renal crisis is a sudden, severe rise in blood pressure with kidney failure. It is a medical emergency, is more likely early in diffuse disease and on steroids, and is treatable if caught. Home blood pressure monitoring is genuinely protective.

Treatment has improved substantially. It is not curable, and organ involvement is now much more manageable than a decade ago.

Signs you might notice

Fingers turning white then blue then red in the cold, with pain and numbness.

Tight, shiny, hardened skin, usually starting at the fingers and face.

Painful ulcers or sores on the fingertips, slow to heal.

A pinched look to the face, and difficulty opening the mouth fully.

Reflux and swallowing difficulty, which are very common. See reflux and dysphagia.

Breathlessness on exertion or a dry cough.

Joint pain, stiffness, and fatigue.

Urgent: a sudden rise in blood pressure with headache or visual change, which may be renal crisis; new or worsening breathlessness; or a finger ulcer that is blackening or infected.

How it can affect day-to-day life

Cold is not a minor inconvenience; it triggers painful attacks and finger ulcers that take months to heal. Heated gloves, layering, keeping the whole body warm and avoiding sudden temperature changes are practical medicine here.

Hand function declines gradually and quietly, and occupational therapy with hand exercises started early preserves a great deal.

Gut involvement causes reflux, bloating, and sometimes malabsorption and weight loss, and needs a dietitian rather than being managed with antacids alone.

Facial and hand changes affect identity and are rarely acknowledged in clinics focused on organ function.

Fatigue is prominent and under-treated.

Supporting someone well

Ask for annual lung function tests and an echocardiogram. This is the screening that changes outcomes and it is sometimes not arranged.

Monitor blood pressure at home, especially in the first years of diffuse disease, and get a sudden rise seen the same day.

Keep the whole body warm, not just the hands. Heated gloves are worth the money.

Get finger ulcers seen promptly; they infect and they are painful.

Ask for hand therapy and daily stretches early.

Ask for a dietitian for reflux, weight loss or bloating.

Ask for referral to a specialist scleroderma centre; care differs measurably.

Stop smoking, which worsens the circulation this condition already compromises.

Where to get help

A GP practice for referral to rheumatology, and a specialist scleroderma centre.

Same-day for a sudden blood pressure rise, new breathlessness, or an infected finger ulcer.

Scleroderma and Raynaud's UK run a helpline and are the specialist charity.