What is Scleroderma

Scleroderma is a chronic, rare autoimmune disease characterized by the hardening and tightening of the skin and connective tissues, often involving internal organ damage due to the overproduction of collagen.

Understanding the Body’s Response

In patients with Scleroderma, the body’s immune system mistakenly attacks its own healthy tissues, triggering an overproduction of collagen. This excess collagen accumulates in the skin, blood vessels, and internal organs, leading to fibrosis (scarring). While the skin changes are the most visible sign, Scleroderma can significantly affect the lungs, heart, kidneys, and digestive tract, requiring a multidisciplinary approach to care.

Classifications and Prevalence

Clinicians generally categorize Scleroderma into two main forms: localized, which primarily affects the skin, and systemic sclerosis, which can involve internal organs. Systemic sclerosis is further divided into limited and diffuse subtypes based on the extent of skin involvement. Current data from the NIH GARD indicates that while exact numbers vary by region, systemic sclerosis affects approximately 75 to 250 per million individuals globally. It is more frequently diagnosed in women than in men, typically appearing between the ages of 30 and 50.

Distinguishing Features

What differentiates Scleroderma from other autoimmune conditions like lupus or rheumatoid arthritis is the specific combination of vascular dysfunction and excessive fibrosis. Many patients experience Raynaud’s phenomenon—a condition where blood vessels in the fingers and toes spasm in response to cold or stress—as one of the earliest signs of the disease. Unlike conditions that cause inflammation without permanent tissue scarring, Scleroderma is defined by the progressive stiffening of tissues, which requires specialized monitoring by rheumatologists.

Medical Disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.

References

• NIH Genetic and Rare Diseases Information Center (GARD)


• Orphanet: The portal for rare diseases and orphan drugs


• Scleroderma Foundation