Scleroderma is a disease that manifests itself by a thickening and hardening of the skin. It mainly affects women from the age of 40.
Scleroderma is a dermatological disease that mainly affects women from the age of 40. “Scleroderma is one of the autoimmune diseases for which there is no perfectly known cause. The immune system produces antibodies against the body’s own constituents“, explains Professor Marie-Sylvie Doutre, dermatologist. The immune system”contribute here to the activation of cells called “fibroblasts” which produce too much of the collagena set of fibrous proteins that modify the structure of the skin and cause sclerosis or fibrosis of the skin. In rare cases, scleroderma can be an occupational disease caused by exposure to silica. Can you die from scleroderma? What impact on life expectancy? Information, photos and treatments.
Definition: what is scleroderma?
“Scleroderma is a disease characterized by thickening of the skin“explains Professor Marie-Sylvie Doutre, dermatologist, straight away. Moreover, scleroderma means “hard skin” in Greek. There are several types of scleroderma. “In some cases, the disease only affects the skin, in the form of hard plaques. This is called localized or morphed scleroderma. In other cases, there may be damage to organs other than the skin: these are the systemic scleroderma of which there are two forms depending on the importance of skin damage : diffuse cutaneous systemic scleroderma and limited cutaneous systemic scleroderma.“
What are the symptoms of scleroderma?
The symptoms of scleroderma are very diverse and multiply as the disease progresses. While localized scleroderma only affects the skin (especially the trunk and abdomen) with a thickening of it which becomes harder, “in systemic scleroderma, it is very often Raynaud’s syndrome which is the first sign: the response of the small vessels of the extremities to various factors, especially cold, is abnormal. There is a decrease in the blood supply to the tissues: fingers and/or toes become white, numb, then blue, tingling, and then red, hot, and painful“, specifies Professor Marie-Sylvie Doutre. Then, the skin damage begins at the level of the fingers and depending on the case, can remain localized at this level or spread to the face, upper and lower limbs, thorax… “Fibrosis and vascular abnormalities can affect organs other than the skin, particularly the digestive tract, lungs, heart, joints, muscles, kidneys.”
Scleroderma: photo of hands
Life expectancy: can you die from scleroderma?
Diffuse cutaneous systemic scleroderma is the most severe form of this disease. the patient’s vital prognosis may be at risk if internal organs such as the lungs, heart or sometimes the kidneys are affected. Eventually, the hardening of the tissues becomes disabling for the patient. “Depending on the case, damage to different organs may appear at the same time or during the course of the disease. Some patients do not have all the possible attacks. The disease is different for each patient given the existence of different forms, from the least serious to the most serious“, specifies Professor Marie-Sylvie Doutre, who adds: “what is important is to have regular medical monitoring which allows treatments to be adapted according to the symptoms and the examinations carried out.“
It is the dermatologist who will make the diagnosis of scleroderma and will follow you up afterwards. Sometimes, however, it will be an internist, a rheumatologist or a pulmonologist who will make the diagnosis based on the symptoms that the patients present.The diagnosis is made by patient questioning to find out what symptoms they present: Raynaud’s syndrome, digestive disorders, shortness of breath, etc. Skin examination also allows the diagnosis of scleroderma“, explains Professor Marie-Sylvie Doutre. A blood test and additional examinations such as a ultrasound of the heart or a study of respiratory function allows us to know if other organs are affected.These tests are carried out at the start of the illness and must be repeated regularly during the monitoring carried out subsequently by the doctor, once or twice a year.“
“There are no treatments to cure systemic scleroderma.but some drugs act effectively on the different manifestations of the disease and can prevent their appearance“, reassures Professor Marie-Sylvie Doutre. Thus, certain symptoms can be treated by immunosuppressants for the most severe forms. In addition, physiotherapy sessions And massages can help improve symptoms, not forgetting adaptation of the environment at home and at work, dietary advice, dental monitoring, etc.Some so-called alternative medicines, such as acupuncture or homeopathymay constitute aid even if their benefits are not demonstrated.“
Thanks to Professor Marie-Sylvie Doutre, dermatologist at the Saint-André Hospital in Bordeaux and member of the French Society of Dermatology (SFD)
