What is scleroderma?
Scleroderma is a rare autoimmune skin condition consisting of hard, thickened skin. This chronic disorder occurs when normal tissue is replaced with excessive collagen, which forms thick, hard tissue that accumulates in the skin and organs. Scleroderma is a dangerous condition as it can form scar tissue on vital organs such as the lungs, heart, gut, blood vessels, and kidneys. The presentation of symptoms for scleroderma varies with each individual but can pose some serious health complications, especially when the heart or lungs may be involved.
Who can develop scleroderma?
Only about 100,000 Americans suffer from this rare condition. Women have a higher tendency to develop scleroderma versus men. It also seems to appear in middle-aged populations around 30-50 years old the most, although there is a pediatric form as well.
Where does scleroderma occur?
Scleroderma is most known to affect the skin but is also able to affect organs as well. Other locations include:
- Gastrointestinal tract
- Blood vessels
What are the different types of scleroderma?
There are several different forms of scleroderma that may present in individuals. The two main types include:
- Localized - the most common type of scleroderma. It usually only impacts the skin in a few locations of the body. However, there are also cases where the joints, bones, and muscles are also affected. Waxy patches or streaks in the skin appear to be the classic presentation found in this type of scleroderma. Symptoms also have the possibility of resolving on their own without intervention.
- Systematic - is the most serious form as it can impact vital organs in the body. It is further divided into two other subgroups.
- Diffuse - this type of scleroderma can be found in many different locations throughout the body and not just in the skin. This form commonly involves the hands and wrists. Vital organs can be affected, making this form potentially life-threatening. Kidney failure and pulmonary hypertension (high pressures in the heart and lung circulation) from scarring of the lungs (pulmonary fibrosis) are the most commonly seen serious complications in this type of scleroderma.
- Limited - most commonly known as CREST syndrome, as each letter represents a characteristic of the condition. It usually presents in the fingers and toes. This form does not involve kidney failure; however, pulmonary hypertension can be found which is also potentially life threatening. Here are the symptoms associated with this type of scleroderma:
- C- represents calcinosis which is an abnormal accumulation of calcium deposits in the skin.
- R- represents Raynaud’s phenomenon.
- E- represents esophageal dysmotility when there is difficulty with swallowing from the condition.
- S- represents sclerodactyly which is tightness and stiffness in the digits.
- T- represents telangiectasia which is red vascular marks in the body.