scleroderma and CREST syndrome
What is it?
Etymologically, it means “hard skin” and is a chronic autoimmune disease that causes thickening of the skin.
The causes of the disease are associated with increased activity of the immune system, leading to damage to the endothelial cells that form the inner lining of blood vessels, and with an increase in the protein that makes up connective tissue, called collagen.
Systemic scleroderma can occur:
- limited form or CREST syndrome (abbreviation for calcinosis, Raynaud’s phenomenon, esophagitis, sclerodactyly, telangiectasia): gradually begins with the first signs of damage mainly to the skin, and then to the gastrointestinal and pulmonary vessels;
- diffuse form: often a rapidly developing form, with symptoms of thickening of the skin, affecting the entire body, as well as internal organs, including the renal arteries.
It is necessary to differentiate the form of scleroderma in which some localized areas of the skin and underlying tissues are affected (focal and linear scleroderma), and the form of systemic scleroderma, with extensive skin lesions, always causing damage to internal organs. Focal scleroderma is characterized by localized and hard skin lesions, often whitish in color, surrounded by a reddish halo. Linear scleroderma is manifested by more frequent linear or striated lesions at the level of the extremities or the back of the head. These forms should be distinguished, since they do not cause damage to the internal organs.
The focal form of the disease can cause both aesthetic problems and problems associated with a serious restriction of the mobility of the joints of the limbs affected by scleroderma. The localized form of scleroderma does not turn into a systematic one
Causes and risk factors
Scleroderma affects adults more often. In women, the disease occurs 4-8 times more often than in men. In particular, women become ill at the perimenopausal age.
The cause is still unknown. In some patients, such syndromes are caused by exposure to certain toxins or bone marrow transplantation.
Which are the symptoms?
The most characteristic signs are thickening and induration of the skin.
Usually, the disease manifests itself on the hands, especially on the fingers (sclerodactyly), and then spreads to other areas. Ulceration of the joints of the fingers and fingertips is common.
On the affected areas of the skin, hair loss is observed, the skin becomes shiny, the subcutaneous layers are compacted, wrinkles gradually disappear and the skin color changes.
Other symptoms:
- telangiectasia (small vessels that become visible on the skin);
- calcification;
- arthritis and muscle weakness;
- difficulty swallowing food;
- delayed peristalsis of the gastrointestinal tract with digestive problems;
- dry eyes and mouth;
- Raynaud’s phenomenon;
- cardiopulmonary lesion;
- kidney damage.
How is it diagnosed?
General laboratory tests are often not very indicative, with antinuclear or anti-centromeric antibodies present.
How is it treated?
There is no radical treatment, but there are measures to combat various aspects of the disease:
- drug therapy: for inflammation and arthralgia, heartburn, Raynaud’s phenomenon and, possibly, for organ damage;
- physical therapy: to maintain and preserve muscle tone and joint function.
More recently, the effectiveness of intravenous administration of prostacyclin analogues has been demonstrated. In some isolated cases, an attempt was made to transplant autologous bone marrow with peripheral stem cells.
The 5-year survival rate from the time of diagnosis is between 80 and 85%. The most serious problems occur due to damage to internal organs, especially the heart, lungs and kidneys.
Where do we treat it?
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