SC131-12 Anatomy and Physiology 2
2015 January 26
“Scleroderma, or systemic sclerosis, is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases” (Scleroderma) Scleroderma means hardening of the skin and that is usually the first sign of this disease. It then moves to other organs. “Scleroderma is relatively rare. Only 75,000 to 100,000 people in the US have it” (American). There is currently two different types of scleroderma, localized and systemic. There are also some subcategories from the main two types.
Localized is just that, localized. It’s found in just a few places on the skin or muscles and typically doesn’t spread to internal organs. Localized scleroderma is also the more mild type. There are two subcategories of localized scleroderma, morphea and linear scleroderma. Morphea is characterized by shiny patches on the skin and are in different shapes and sizes. The skin under these patches may be thicker. The patches can come and go and can change their shape. This form is usually seen in children, but also appears in adults from ages 20-50. Linear scleroderma comes in stripes or lines typically on the arms, legs, or forehead. Linear scleroderma can go into deeper layers of skin than morphea does. Because of that, this type can also hinder mobility in those areas. Linear scleroderma is seen in children more than adults.
Systemic scleroderma, which can also be referred to systemic sclerosis, is much more complex. “The changes occurring in systemic scleroderma may affect the connective tissue in many parts of the body. Systemic scleroderma can involve the skin, esophagus, gastrointestinal tract (stomach and bowels), lungs, kidneys, heart and other internal organs. It can also affect blood vessels, muscles and joints” (Scleroderma). The tissue in these organs become hard causing them to not function as normal. There is also two subcategories of systemic scleroderma. The first is diffuse scleroderma. This is when the skin thickening happens quicker and is in a much larger area. People with the type have a higher chance of it spreading to the internal organs. The second subcategory is limited scleroderma. This kind doesn’t progress as fast and is more confined to the fingers, hands, and face. Internal problems are less common for this type compared to diffuse scleroderma. Both these types can cause pulmonary hypertension, which is a condition that limits blood flow through the lungs and results in shortness of breath.
Symptoms with scleroderma typically begin right away. The first sign for most people is skin hardening, which can appear in stripes or circular patches, depending which type a person has. This can vary from patient to patient. The skin may also appear to be shiny or have restricted movement in the hardened areas. Another first sign is an over exaggerated respond to cold temperatures or emotional distress. This can cause numbness or pain and can cause the fingers and toes to change colors. This condition is called Raynaud's phenomenon. There’s also some digestive issues that can start to show. Acid reflux may develop and it also may be harder to absorb nutrients, because the muscles may not push food through properly. Lastly, although rare, issues with function of the heart, lungs, and kidneys can happen and can be life threatening. Because most rheumatoid and autoimmune diseases have the same symptoms, scleroderma is sometimes hard to diagnose and many patients receive a misdiagnosis at first.
The direct cause for scleroderma is still unknown. “Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin” (Mayo Clinic). Doctors aren’t sure what causes this overproduction, but the immune system has something to do with it. It seems that the immune systems attacks