What is scleroderma?
Scleroderma (sklair-oh-DUR-muh), also known as systemic sclerosis, is a group of rare diseases that involve the hardening and tightening of the skin. It may also cause problems in the blood vessels, internal organs and digestive tract.
Scleroderma is often categorised as “limited” or “diffuse,” which refers only to the degree of skin involvement. Both types can involve any of the other vascular or organ problems. While there is no cure for scleroderma, treatments can ease symptoms, slow progression and improve quality of life.
Forms of scleroderma
There are 2 major forms of scleroderma, localized and systemic. Systemic scleroderma can be broken down into two main types: diffuse and limited.
The more common form of the disease, localized scleroderma, affects only a person’s skin, usually in just a few places. It often appears in the form of waxy patches or streaks on the skin, and it is not uncommon for this less severe form to go away or stop progressing without treatment.
As its name implies, this form affects many parts of the body. Not only can it affect the skin, but it also can affect many internal organs, hindering digestive and respiratory functions, and causing kidney failure. Systemic scleroderma can sometimes become serious and life-threatening.
Also known as CREST syndrome, each letter stands for a feature of the disease:
- C alcinosis (abnormal calcium deposits in the skin)
- R aynaud’s phenomenon (see the symptoms section)
- E sophageal dysmotility (difficulty swallowing)
- S clerodactyly (skin tightening on the fingers)
- T elangectasias (red spots on the skin)
Patients with limited scleroderma do not experience kidney problems. The skin thickening is restricted to the fingers, hands and forearms, and also sometimes the feet and legs. Digestive involvement is confined mostly to the oesophagus. Among later complications, pulmonary hypertension, which can develop in 20% to 30% of cases, can be potentially serious. In pulmonary hypertension, the arteries from the heart to the lungs narrow down and generate high pressure on the right side of the heart, which can ultimately lead to right sided heart failure. Early symptoms of pulmonary hypertension include shortness of breath, chest pain, and fatigue.