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Systemic scleroderma
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Condition

Systemic scleroderma

Systemic scleroderma is an autoimmune disorder characterized by the buildup of fibrosis in the skin and internal organs due to the excess production of collagen.

Overview

In systemic scleroderma, the immune system malfunctions and attacks the body's own tissues. The resulting fibrosis (thickening and hardening of tissue) can affect the skin and vital organs, including the esophagus, heart, lungs, and kidneys.

Common Signs and Symptoms

  • Raynaud phenomenon: Fingers and toes turning white or blue in response to cold or stress.
  • Skin changes: Puffy hands, followed by thickening and hardening (sclerodactyly).
  • Dermal features: Open sores (ulcers) on fingers, painful bumps (calcinosis), or enlarged blood vessels (telangiectasia).
  • Internal involvement: Heartburn, difficulty swallowing, shortness of breath, or kidney problems.

Types

  • Limited cutaneous systemic scleroderma: Fibrosis typically affects only the hands, arms, and face.
  • Diffuse cutaneous systemic scleroderma: Fibrosis affects large areas of skin, including the torso, and often involves internal organs with rapid progression.
  • Systemic sclerosis sine scleroderma: Fibrosis affects internal organs but does not involve the skin.

Causes

The exact cause is unknown, but variations in several genes—particularly those in the HLA complex—influence risk. Other genes, such as IRF5 and STAT4, which are involved in immune response, are also associated with the condition. Genetic factors likely interact with environmental triggers to determine the risk of developing the disorder.

Inheritance

Most cases are sporadic, occurring in people with no family history of the condition. While it can occasionally run in families, there is no clear pattern of inheritance. Inheriting a genetic variation linked to systemic scleroderma does not guarantee that an individual will develop the condition.

Frequently Asked Questions

Yes. Smoking cessation, regular low‑impact aerobic exercise, and maintaining a warm environment reduce vascular injury. A low‑salt, low‑acid diet (limiting citrus, coffee, chocolate, and spicy foods) can lessen reflux. Daily skin moisturizers with urea or ceramides improve elasticity. Regular follow‑up with a multidisciplinary team (rheumatology, pulmonology, cardiology, gastroenterology, nephrology) allows early detection and treatment of organ involvement, which is the most effective way to slow disease progression.
Raynaud phenomenon is common, but when it is accompanied by skin thickening (especially on the fingers or face), new digital ulcers, or telangiectasias, clinicians become suspicious of systemic sclerosis. A rheumatologist will look for these skin changes, order antinuclear antibody (ANA) testing and specific autoantibodies (e.g., anti‑centromere, anti‑Scl‑70) to confirm the diagnosis.
Sudden shortness of breath, new or worsening heartburn that does not improve with medication, unexplained swelling of the legs, rapid weight gain, or a sudden rise in blood pressure (especially if accompanied by headaches or visual changes) can signal lung, gastrointestinal, cardiac, or renal involvement. These require prompt evaluation, often in an emergency or urgent‑care setting.
Keep the area clean with mild soap and water, apply a non‑adhesive sterile dressing, and avoid tight gloves or cold exposure that worsens Raynaud. Topical antimicrobial ointments may be advised by your doctor. For calcinosis, gentle massage is not recommended; instead, discuss options such as calcium‑binding agents or surgical removal if the deposits cause pain or recurrent ulcers.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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