The Scleroderma is a disease that causes a thickening of the connective tissue exaggerated and skin of the entire body, giving it a stiff appearance.
This condition is different from other pathologies such as dermatitis or thickening of the skin that is evidenced in hypothyroidism and chronic kidney disease, since in scleroderma the hardening of the connective tissue can be so severe that it can compromise internal organs such as blood vessels, esophagus and kidneys.
When scleroderma affects exclusively the skin, it is defined as localized, and when it affects internal organs, it is called systemic sclerosis.
If you want to know more about this interesting condition, this article will explain how it occurs, the types and what are the symptoms of scleroderma.
Why does scleroderma occur?
The scleroderma is autoimmune origin, this means that the body can produce autoantibodies that attack the skin and all that type of connective tissue found inside of an organ. That is why it can be associated with other autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, etc.
In some cases, some chemicals have been identified that could trigger the appearance of this picture, among them: pesticides, resins and organic solvents in some paints.
Classification of scleroderma
There are different ways to classify scleroderma, listed below:
Linear or “saber-bodied” scleroderma
It is a type of scleroderma common in children, it is characterized by a localized thickening of the skin with a linear distribution, predominantly affecting the patient's head and neck in a progressive manner, even compromising their internal organs such as the brain (leading to headache and seizures).
Morphea scleroderma
It is the type of scleroderma that is distributed in the form of patches on the upper limbs, chest, hands and face. It can be localized or generalized.
The scleroderma morphea may vary from millimeter lesions (morphea punctate) to affect the subcutaneous tissue (deep morphea), but unlike systemic sclerosis respects blood vessels and internal organs.
Systemic sclerosis
In this type of scleroderma there is involvement of the skin together with alterations of the internal organs. It is commonly associated with other autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, and dermatomyositis.
There is a form of systemic sclerosis called CREST Syndrome (for its acronym in English), which is characterized as a form of scleroderma, associated with:
- C alcinosis: accumulation of calcium in the skin and joints.
- R aynoud phenomenon: fingers that turn purple when exposed to very cold temperatures, can occur on the fingertips or the entire finger.
- Dysmotility and sofágica: manifested by difficulty swallowing food.
- E s clerodactyly: characterized by thin and hardened fingers, which makes mobility difficult.
- T elangiectasias: spider veins on the skin, associated with the vascular condition of the disease.
There is a form of diffuse cutaneous scleroderma where, in addition to skin symptoms, there is kidney, heart and lung damage.
Scleroderma symptoms
The symptoms will depend on the type of scleroderma that develops in the patient. However, in general, patients with scleroderma have the following symptoms:
- Thickening of the skin (stiff skin) in delimited or generalized areas, distributed in the hands, arms, face, thorax and feet.
- Limitation to open the mouth.
- Fingertips thinned due to loss of connective tissue.
- Purple coloration of the fingers in some cases (Raynaud's phenomenon).
- Ulcers and injuries in the hands due to compromise of the blood vessels of the fingers in some cases.
- Cutaneous calcinosis that is due to the accumulation of calcium in the skin.
- Rubbing sound when moving some joints due to friction of tendons in wrists, elbows and heels.
- Difficulty breathing, fainting and tachycardia in case of involvement of large vessels of the heart and lung (in pulmonary artery hypertension and pulmonary fibrosis).
Diagnosis:
The diagnosis is made with a thorough physical examination that not only evaluates the changes in the skin, but also allows one to suspect an underlying organic lesion.
The doctor should indicate the detection of antibodies in the blood, including ANA (antinuclear antibody) that is positive in more than 95% of cases of cutaneous systemic sclerosis. In addition, a skin biopsy is performed and complementary studies such as kidney function, chest X-ray, esophageal studies, and cardiac and respiratory tests are requested, if deemed necessary.
Treatment
At present there is no treatment to reverse the symptoms of scleroderma once it is established, for which treatment is indicated to avoid its progression and the vascular and organic complications of the disease.
Among the drugs used are steroids, mycophenolate mofetil and cyclophosphamide, which are indicated to regulate the immune response. In addition, medications are used that improve blood flow and respiratory symptoms depending on the organs affected.
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