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What is Systemic Sclerosis? The Skin-Hardening Disease Explained
Published: Mar 05, 2024
Systemic sclerosis, also known as scleroderma, is a complex disease that affects the skin and internal organs. It causes the skin to become thick and hard, and can lead to serious complications throughout the body.
More Than Just Skin Deep
While skin thickening is the hallmark of systemic sclerosis, it's not just a skin condition. The disease can affect blood vessels, muscles, joints, and internal organs like the lungs, heart, and kidneys. This widespread impact is why it's called 'systemic' sclerosis.
Types of Systemic Sclerosis
There are two main types of systemic sclerosis: limited and diffuse. Limited systemic sclerosis affects the skin on the face, hands, and lower arms and legs. Diffuse systemic sclerosis is more severe, affecting larger areas of skin and often involving internal organs. Some people may have systemic sclerosis without skin involvement, called 'sine scleroderma'.
Who Gets Systemic Sclerosis?
Systemic sclerosis is rare, affecting between 38 and 341 people per million worldwide. It's more common in women, with a female to male ratio ranging from 3:1 to 8:1. The disease can occur at any age but typically starts between ages 30 and 50.
Frequently Asked Questions
Currently, there's no cure, but treatments can manage symptoms and slow progression.
The exact cause is unknown, but it involves autoimmunity, vascular problems, and fibrosis.
There's a genetic component, but it's not directly inherited.
In severe cases, it can be life-threatening due to organ complications.
Living with Systemic Sclerosis
While systemic sclerosis is a challenging condition, many people with the disease lead full, productive lives with proper management and care.
References
- Allanore Y, Simms R, Distler O, et al. Systemic sclerosis. Nat Rev Dis Primers 2015; 1:15002.
- Ingegnoli F, Ughi N, Mihai C. Update on the epidemiology, risk factors, and disease outcomes of systemic sclerosis. Best Pract Res Clin Rheumatol 2018; 32:223.
- van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 2013; 72:1747.
This article has been reviewed for accuracy by one of the licensed medical doctors working for Doctronic. Always discuss health information with your healthcare provider.
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