Scleroderma, also known as systemic sclerosis, is a rare autoimmune disease that affects the connective tissues in the body. Connective tissues are the glue that holds everything together, providing structure and support to organs, skin, and muscles. In scleroderma, the body’s immune system mistakenly attacks these tissues, leading to an overproduction of collagen, a protein that gives structure to the skin. This excess collagen builds up, causing the skin to harden and tighten. Scleroderma can also affect internal organs, leading to a variety of complications.
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Types of Scleroderma
There are two main types of scleroderma:
- Localized scleroderma: This type only affects the skin and usually doesn’t involve internal organs. It’s further classified into three subtypes:
- Morphea: This is the most common form of localized scleroderma, causing patches of thickened, hardened skin.
- Linear scleroderma: This type affects a band of skin, typically on the arm, leg, or forehead.
- Scleroderma en coup de sabre (French for “scimitar stroke”): This subtype causes a hardened, linear patch of skin on the forehead or scalp.
- Systemic sclerosis: This is the more severe form of the disease, affecting not only the skin but also internal organs like the lungs, heart, kidneys, and digestive system. Systemic sclerosis can be further divided into two subtypes:
- Limited cutaneous systemic sclerosis (formerly known as CREST syndrome): This type primarily affects the hands, feet, and face. CREST stands for Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, https://emedicine.medscape.com/article/1064663-overview?form=fpfSclerodactyly (thickened skin of the fingers), and Telangiectasia (visible dilated blood vessels).
- Diffuse cutaneous systemic sclerosis: This type involves widespread thickening of the skin, not just the hands and feet. It’s generally more aggressive and can cause significant internal organ damage.
Symptoms of Scleroderma
The symptoms of scleroderma vary depending on the type and severity of the disease. Some common symptoms include:
- Skin thickening and hardening: This is the most common symptom, affecting all types of scleroderma. The skin may become tight, shiny, and difficult to move.
- Raynaud’s phenomenon: This is a condition that causes fingers and toes to become numb, tingly, and change color (red, white, or blue) in response to cold temperatures or emotional stress.
- Joint pain and stiffness: Scleroderma can cause inflammation and stiffness in the joints.
- Fatigue: Many people with scleroderma experience chronic fatigue.
- Digestive problems: Scleroderma can affect the digestive system, leading to problems like heartburn, difficulty swallowing, bloating, and constipation.
- Breathing problems: In severe cases, scleroderma can affect the lungs, causing shortness of breath, coughing, and difficulty exercising.
Causes of Scleroderma
The exact cause of scleroderma is unknown. However, several factors are believed to play a role, including:
- Autoimmune system: In scleroderma, the immune system mistakenly attacks the body’s own tissues.
- Genetics: Having a family history of autoimmune diseases slightly increases the risk of developing scleroderma.
- Environmental triggers: Exposure to certain chemicals or toxins may trigger scleroderma in people who are genetically predisposed. However, this link is not fully understood.
Diagnosis of Scleroderma
There’s no single test to diagnose scleroderma. Doctors typically rely on a combination of factors, including:
- Medical history and physical examination: The doctor will ask about your symptoms and perform a physical exam to look for signs of skin thickening, Raynaud’s phenomenon, and joint problems.
- Blood tests: Blood tests can help identify certain antibodies that are often present in people with scleroderma.
- Imaging tests: X-rays, CT scans, and MRIs can help assess internal organ involvement.
Treatment of Scleroderma
There’s no cure for scleroderma, but there are treatments that can help manage the symptoms and slow the progression of the disease. Treatment options may include:
- Medications: Medications like corticosteroids and immunosuppressants can help suppress the immune system and reduce inflammation.
- Physical therapy: Physical therapy can help maintain flexibility and joint function.
- Occupational therapy: Occupational therapy can help you learn techniques to manage daily activities with limitations caused by scleroderma.
