NG-316

🦵 Scleroderma

An autoimmune disease that hardens connective tissue. Skin is what you see; the organs are what kills.

🧬 What it is

A hand drawn three times in the three colors of a Raynaud attack - white, then blue, then red as the blood floods back - with CREST beside it, and the renal crisis that is an emergency. — Raynaud only makes sense in color: white as the vessels clamp, blue as the tissue starves, red and burning as the blood comes back. Swipe it sideways if it is cut off, or tap to open it full size.

The immune system drives overproduction of collagen. Tissue that should be soft and elastic becomes thick, tight and fibrotic.

Scleroderma literally means "hard skin" - but the same hardening happens in the lungs, kidneys, heart and gut, and that is what determines survival.

Type	Skin involvement	Organs
Localized	Patches only (morphea, linear)	Spared
Limited (CREST)	Hands, forearms, face	Slower; pulmonary hypertension later
Diffuse systemic	Widespread, rapid	Lungs, kidneys, heart, GI — early

⭐ CREST — the limited form

Calcinosis — calcium deposits under the skin
Raynaud’s phenomenon — usually the first symptom, often years ahead
Esophageal dysmotility — reflux, difficulty swallowing
Sclerodactyly — tight, shiny, claw-like fingers
Telangiectasia — small dilated surface vessels

👀 What you see

Skin — tight, shiny, hard; loss of wrinkles; mask-like face with reduced mouth opening
Hands — puffy early, then sclerodactyly; contractures; difficulty gripping
Raynaud’s — fingers go white → blue → red with cold or stress
GI — severe reflux, dysphagia, early satiety, constipation
Lungs — exertional dyspnea, dry cough — the leading cause of death
Kidney — sudden severe hypertension (renal crisis)

🚨 Scleroderma renal crisis

Abrupt malignant hypertension with rapidly rising creatinine. A true emergency, treated with ACE inhibitors.

A sudden blood pressure spike in a scleroderma patient is renal crisis until proven otherwise. Report immediately - do not treat it as routine hypertension.

🩺 Nursing care

🌡️ Raynaud’s — the daily reality

Keep warm — gloves, layers, warm the car before driving
Avoid cold, including the frozen aisle and cold drinks
Stop smoking — nicotine constricts vessels; this is non-negotiable
Manage stress — it triggers attacks
Inspect fingertips daily for ulcers; report early
Calcium channel blockers (nifedipine) are commonly prescribed

🍽️ Esophageal involvement

Small frequent meals; soft, moist food
Sit upright for 1–2 hours after eating; do not eat before bed
Raise the head of the bed on blocks
Proton pump inhibitors, usually long term

Untreated reflux in scleroderma causes strictures and aspiration. Positioning is treatment, not comfort.

🦵 Skin and joints

Moisturise generously; avoid harsh soaps and very hot water
Range-of-motion exercises daily to delay contractures — this is the intervention that preserves function
Protect from injury; healing is poor
Occupational therapy for adaptive tools — grip aids, button hooks

🎯 NCLEX traps

Raynaud’s is usually the first sign, often years before diagnosis
Lung disease is the leading cause of death, not the skin
A sudden BP spike = renal crisis → ACE inhibitor
Warmth and smoking cessation are the core teaching
Daily ROM prevents the contractures that cause disability

Sources. Written from MedlinePlus, NIAMS, National Cancer Institute and OpenStax A&P 2e (CC BY 4.0).