Scleroderma

Scleroderma is a rare autoimmune disease characterized by thick and tightening skin. It produces an accumulation of scar tissue and causes damage to organs such as the heart, lungs, kidneys and stomach. In some aspects it has similarities with lupus and rheumatoid arthritis. Autoimmune diseases (those in which the body attacks itself) are becoming more frequent.

Scleroderma is frequent among middle-aged women between 30 and 50, although it can occur at any age. Currently, the exact causes of scleroderma are unknown. However, it is known that besides its relation to the immune system, it’s also connected to microcirculatory disorders and perivascular inflammatory infiltrates, which are related to immune activation, antibody production and an excessive presence of fibroblasts (cells responsible for synthesizing other cells that form the connective tissue). Among the factors that originate these processes, current research points to an influence of genetic inheritance and environment, more specifically, the exposure to toxic agents, oxidative stress and free radicals.

Types of Scleroderma

There are different types of scleroderma that present different prognoses. However, all these different types can be classified into two large groups:

  • Localized Scleroderma:

Is characterized by affecting only the skin, without the occurrence of Raynaud’s phenomenon, resulting in a better prognosis.

  • Systemic Scleroderma:

Besides presenting a cutaneous affection, it causes visceral damage and thus, a worse prognosis.

The symptoms of scleroderma vary from person to person, depending on the severity and type. Some of the possible symptoms are:

  • Progressive tightness of the skin: this is the most common sign of scleroderma. During a variable number of years, the affected person will show hardening of the skin. This hardening occurs especially in the hands (sclerodactyly), accompanied by Raynaud’s phenomenon.

  • The phenomenon of Raynaud: it is a vascular disorder characterized by a lack or reduction of blood supply to the fingers (ischemia) in an episodic way. It manifests an alteration of the color of the hands after exposure to cold and redness after exposure to heat.

  • Numbness of the fingers and toes: this sign constitutes an exaggerated response to cold, causing numbness or pain accompanied by changes in color.

  • Problems of the digestive system: the gastrointestinal issues affect mainly the esophagus. However, any part of the digestive tract may suffer alterations.

  • Pulmonary complications: patients with scleroderma have a difficulty breathing. Also, in general, they suffer a gradual deterioration of lung function as a result of fibrosis. This is the main cause of death in these patients.

  • Cardiac issues: this situation occurs rarely and manifests itself mainly in the form of arrhythmias and angina-type chest pain.

  • Malfunction of the kidneys: when it affects the kidney, its called nephropathy (any disease that affects the kidney), and appears during the first years of the disease.

Other symptoms of this disease include joint pain, hair loss, dry cough and wheezing (sound made by air passing through congested airways).

If you suspect that you may have scleroderma, you should see a doctor to perform medical tests. For diagnosis, your doctor can request a blood test (to detect the presence of antibodies), a capillaroscopy (allows visualization of the microscopic vessels of the skin) and/or a cutaneous biopsy (consists of taking a piece of skin to analyze it under a microscope).

Currently, there is no allopathic treatment (based on medications) that can cure the disease. Because it is a heterogeneous condition that can present diverse symptoms, treatment must be adapted to each patient individually. There are certain symptoms that can be treated with immunosuppressive medication in more severe cases. These symptomatic treatments are key to improve the prognosis of the disease.

Treatment can also be complemented with non-medicated care. An example is physiotherapy, which helps maintain joint mobility. Because the skin becomes fragile, it will be necessary to protect it from the cold and the sun. It is recommended to apply moisturizers adapted to the needs of your skin.

Some other simple measures can be put into practice to attenuate the symptoms. These measures would be aimed at improving general health of the patient, such as smoking cessation, doing physical exercise on a regular basis (walking or swimming) and a healthy diet in order to maintain an ideal weight.

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