Scleroderma ICD-10: M34.9

Autoimmune / Connective tissue disease

Often searched as: skin tightening condition, hard skin on hands and face, fingers changing color in cold, Raynaud's cold hands, stiff tight skin autoimmune, skin hardening disease, shiny tight skin hands, autoimmune skin and organs…

Clinical urgency level

lowmediumhigh

Systems Affected

skinlungsheartkidneysgastrointestinal tractblood vesselsjoints

Severity Levels

mild

Localized scleroderma (morphea or linear) — affects skin only, no internal organ involvement, generally manageable.

moderate

Limited cutaneous systemic sclerosis (lcSSc) — skin thickening on hands and face, Raynaud's phenomenon, possible esophageal involvement; slower progression.

severe

Diffuse cutaneous systemic sclerosis (dcSSc) — rapid skin thickening spreading to trunk, with major organ involvement (interstitial lung disease, pulmonary hypertension, renal crisis); highest risk of complications.

Red Flags

  • Fingers turning white, blue, or red with temperature changes (Raynaud's) — especially if new
  • Rapidly thickening or tightening skin on hands, face, or arms
  • Shortness of breath or dry cough (possible lung involvement)
  • Difficulty swallowing or persistent heartburn (esophageal involvement)
  • Sudden high blood pressure and kidney changes (scleroderma renal crisis — emergency)
  • Digital ulcers or non-healing sores on fingertips

Clinical Presentation

Scleroderma — tight, shiny skin on hands with sclerodactyly

Scleroderma — tight, shiny skin on hands with sclerodactyly

Source: Wikimedia Commons (CC BY-SA 3.0 )

Scleroderma facial features — tightened perioral skin

View gallery on DermNet NZ (image gallery reference)

When to See a Doctor

See a rheumatologist or dermatologist if your skin is progressively tightening, especially on the hands or face, or if you experience Raynaud's phenomenon alongside other symptoms. Shortness of breath, swallowing difficulties, or a sudden blood pressure spike in a known scleroderma patient require emergency evaluation.

Differential Diagnosis

  • Morphea (localized scleroderma — skin only)
  • Mixed connective tissue disease
  • Systemic lupus erythematosus
  • Dermatomyositis
  • Eosinophilic fasciitis
  • Nephrogenic systemic fibrosis
  • Chronic graft-versus-host disease

Comorbidities

  • Raynaud's phenomenon (in nearly all patients)
  • Pulmonary arterial hypertension
  • Interstitial lung disease
  • Gastroesophageal reflux disease (GERD)
  • Sjogren's syndrome
  • Hypothyroidism
  • Depression and anxiety

Prognosis

Prognosis varies widely by subtype. Localized scleroderma rarely affects internal organs and has a good prognosis. Limited systemic sclerosis progresses slowly and many patients live decades with good quality of life. Diffuse systemic sclerosis carries a more serious prognosis due to rapid organ involvement — pulmonary hypertension and lung fibrosis are the leading causes of death. Early diagnosis and disease-modifying treatment significantly improve outcomes. There is no cure, but management options have advanced considerably.

Detailed Overview

Causes, Symptoms, and When to Take Action Scleroderma (also called systemic sclerosis) is a connective tissue disease characterized by overproduction of collagen, which leads to hardening and thickening of the skin and possibly internal organs. How It Looks Texture: Skin becomes tight, thick, and shiny; may lose normal elasticity Color: Areas with excessive collagen may appear lighter or waxy Shape: Skin changes often begin in limited areas (hands, face) and may spread Location: Typically affects the skin of the hands, face, arms; in systemic forms, internal organs (lungs, heart, digestive tract) may also be involved Why Does It Appear? The disease is autoimmune in nature: the immune system triggers excessive collagen production Blood vessels may also be damaged, contributing to reduced blood flow and tissue changes The exact cause is unknown; genetic predisposition and environmental triggers may play a role Should You Be Concerned? Yes. While localized forms affect the skin only, systemic scleroderma can involve internal organs and lead to complications: Progressive changes in lungs, heart, kidneys, or digestive system Symptoms such as difficulty breathing, swallowing, or blood pressure issues Early recognition is critical to manage potential organ involvement Can You Prevent It? There's no known way to prevent scleroderma. However: Monitoring for early signs may allow timely intervention Protecting skin and avoiding trauma may reduce severity of skin changes 👉 How Piel AI Can Help Piel AI allows you to follow skin changes over time via images. While not diagnostic, it helps you notice patterns of thickening or progression that should prompt medical evaluation. Summary Scleroderma is a connective tissue disease characterized by collagen overproduction, leading to skin and possibly organ involvement. Skin becomes tight, thickened, and less flexible; internal organs may be affected in systemic forms. The cause is autoimmune; triggers and genetics may influence onset. Early monitoring and evaluation are essential due to possible internal complications.

Tags

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