Scleroderma is an autoimmune disease. This means that it is a condition that causes the body’s immune system attacks its own tissues. Scleroderma may vary a lot in terms of severity. In some cases, it can be a mild condition, while in others it can be a life-threatening situation.
What Are The First Signs Of Scleroderma?
The most common symptom of scleroderma disease is a hardening or tightening of patches of skin, however, one of the first signs of scleroderma is an exaggerated reaction to cold temperatures or emotional distress, which can cause numbness, pain or discoloration of the fingers or toes. This condition, also called Raynaud’s disease, also occurs in people who do not have scleroderma.
There are two main types of scleroderma: the systemic (systemic sclerosis, SSc), which usually affects the skin and the internal organs or systems of the body, and the localized scleroderma.
What is Systemic Sclerosis?
There are two main types of systemic sclerosis or SSc, for its acronym in English: the limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis. The difference between limited and diffuse systemic cutaneous sclerosis lies in the extent of the affected skin. In the limited cutaneous systemic sclerosis, the thickening of the skin occurs only in the hands and forearms, feet and lower part of legs. In contrast, diffuse cutaneous systemic sclerosis affects the hands, the forearms, the upper arms, the thighs or the trunk. Both modalities can affect the face.
Both limited and diffuse cutaneous systemic sclerosis may have serious clinical characteristics. For example, the phenomenon of Raynaud is presented in both modalities of the disease. The Raynaud phenomenon is a condition which causes the skin of the fingers to turn white or blue during exposure to cold and then turn red or purple when it recovers the heat. These episodes are caused by a spasm of the small blood vessels that are in the fingers. Over time, these small blood vessels they suffer damage to the point that they can remain completely clogged, this can make them appear ulcerations on the fingertips. The phenomenon of Raynaud is presented in almost all patients with systemic sclerosis (95%).
Almost all patients with systemic sclerosis also have affected the esophagus, which loses normal movement. In consequence, the food can “get stuck” in the esophagus and the stomach acid may go up into the esophagus and cause heartburn.
Telangiectasias are small red dots that appear in the hands, arms, face or trunk. They are tiny blood vessels of the skin that have dilated.
Patients affected by diffuse cutaneous systemic sclerosis are at increased risk of developing pulmonary fibrosis disease or interstitial lung disease (scars on the lung tissue that make breathing difficult), kidney disease and intestinal disease.
Renal involvement occurs and usually manifests as a sudden increase in blood pressure.
The risk of extensive bowel involvement, with slowing of movements or motility of the stomach and intestines origin the following symptoms: the sensation of swelling after eating, diarrhea or periods of constipation alternated with periods of diarrhea.
Calcinosis refers to the presence of calcium deposits in the skin or just below it.
Pulmonary hypertension is high blood pressure in the blood vessels of the lungs. The most frequent symptom is shortness of breath (difficulty breathing) when an effort is made.
What is Localized Scleroderma?
Morphea manifests as plaques or patches of thickened skin that can be from half an inch to six inches in diameter. Some people only have one or a few plates, while others have several scattered throughout the body. The plaques may be lighter or darker in color than the surrounding skin and, therefore, they usually stand out.
Linear scleroderma manifests as a strip of thickened skin along with an arm or side of a leg. The subcutaneous layer of fat can be lost so that the affected limb is thinner than the one that is not.
Scleroderma in coup de saber
Scleroderma in coup de saber is a modality of linear scleroderma, in which the thickened skin line appears on the forehead or on another part of the face.