What is it about ?
The term ‘scleroderma’ comes from the Greek ‘scleros’ (hardening) and ‘derma’ (skin). Scleroderma is characterized by stiffness and hardening (sclerosis) of the skin and other organs due to an increase in connective tissue. This condition is also called systemic sclerosis because it also affects internal organs (joints, blood vessels, etc.). It is an autoimmune disease: the body makes antibodies against itself. The cause is not yet well known.
What is its frequency?
Scleroderma affects only 1 to 2 in 10,000 people. It is more common in women between the ages of 30 and 50.
How to recognize it?
In scleroderma, 9 times out of 10, it is the Raynaud’s phenomenon which is the first symptom. In some (severe) cases, the condition shows ulcers and scars. Muscle and / or joint pain occurs in 2 to 3 in 10 people affected.
There are 2 forms of scleroderma:
- the generalized scleroderma is characterized by stiffness and hardening of the skin which often changes rapidly and gives a shiny appearance to the skin. The hardened skin gradually prevents the smooth movement of the joints. Wrinkles disappear and the nose looks sharper. As the skin is much stiffer, the opening of the mouth may be limited. Organs such as the heart, kidneys, lungs or digestive tract are affected, causing increased blood pressure, fatigue, shortness of breath on exertion, a dry cough, palpitations or heart failure, inflammation of the lining of the heart (pericarditis) and some constipation. A severe kidney attack can occur, as well as high blood pressure, rapid deterioration of kidney function, and severe kidney failure. anemia.
- the limited scleroderma is limited to the skin of the fingers, forearms, feet, thighs and face. The mobility of the esophagus can also be disturbed. The consequences are problems with swallowing (dysphagia) and burning (gastroesophageal reflux). Kidney damage or lung damage is rare.
- in case of localized scleroderma, only the skin is affected.
- Sometimes sclerosis in the fingers is part of a larger syndrome: CREST syndrome. “CREST” is an acronym which designates the appearance, at the same time, of a VSalkinosis (accumulation of calcium salts in the tissues), the phenomenon of Raynaud, complaints about the (o)Esophagus), a Sclerodactyly (hardening of the skin on the fingers) and Telangiectasias (small red spots on the skin (macules) caused by vasodilation).
How is the diagnosis made?
The doctor will suspect the diagnosis based on the clinical picture. He will ask for a complete blood test including the detection of specific antibodies (in particular antinuclear antibodies and rheumatoid factor) and inflammatory parameters (CRP, sedimentation).
Sometimes a skin biopsy is needed to confirm the diagnosis. A special examination of the blood vessels allows the doctor to detect changes characteristic of the small blood vessels at the edge of the nails. This examination is also useful for the follow-up of the patient afterwards.
It is also possible to call on a specialist (rheumatologist) for diagnosis and follow-up.
What can you do ?
Even more than other people, if you have scleroderma, you need to protect your skin and avoid the cold. It is perfectly appropriate to quit smoking.
What can your doctor do?
Scleroderma cannot be cured. Cortisone, vasodilator drugs, and drugs that slow down the activity of the immune system help keep the disease under control. Anti-inflammatory drugs relieve pain in muscles and joints. In case of burning, the doctor will prescribe medication against acidity. He will favor the taking of hypotensive drugs in the event of arterial hypertension or renal crises. He will regularly monitor blood pressure and kidney function.
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