Scleroderma is a rare and chronic condition that causes connective tissue, which holds cells together and supports different organs, to become abnormally thick and hard. The condition typically affects the skin and blood vessels, but also can affect internal organs such as the heart, lungs, kidneys, and intestinal tract.
The symptoms of scleroderma can vary dramatically, depending on which organs are affected and how severely. For some patients, symptoms can be quite mild; for others, they can be life-threatening.
Localized versus systemic scleroderma
In general, localized scleroderma (also known as morphea) primarily affects the skin, rarely damages internal organs, and runs a milder course. Localized scleroderma can be further classified into plaque morphea, bullous morphea, deep morphea, generalized morphea, and linear scleroderma — based on areas of the skin being affected.
Systemic scleroderma is the more serious of the two types. Patients may experience a broad range of symptoms due to damage to the internal organs. Systemic scleroderma is categorized into limited and diffuse cutaneous scleroderma, with the latter being the more serious of the two.
The type of scleroderma that a patient also has affects the speed at which symptoms develop.
The name scleroderma comes from the words sclero — thickening — and derma — skin. True to its name, nearly all patients with scleroderma have patches or streaks of thick and hardened skin. Affected areas also may be discolored and lose hair.
Other skin problems linked to scleroderma include sclerodactyly (tight skin over fingers that causes them to curl inward), microstomia (a smaller mouth opening due to tight skin around the mouth, causing difficulties in eating, brushing, flossing, and speaking), calcinosis (white, painful lumps under the skin), digital ulcers, dry skin (also called xerosis) that can be itchy or tingling, and unusual patches of darkened or lightened skin.
Involvement of other body systems
Different parts of the body can be affected by scleroderma and cause a range of other symptoms. These are listed below:
- Blood vessels: damage to blood vessels can cause a condition called Raynaud’s phenomenon, which can result in telangiectasias and gangrene in severe cases.
- Muscles and bones: damage to muscle and bone tissue can cause pain and muscle weakness.
- Lungs: damage to lungs can cause shortness of breath, a dry cough, and chest pain.
- Heart: damage to the heart can cause an abnormal heart rhythm and symptoms of heart failure.
- Gastrointestinal tract: damage to the gastrointestinal tract can cause bloating, nausea or vomiting, a burning sensation in the chest (heartburn), difficulty swallowing, stomach pain, weight loss, diarrhea, and constipation.
- Eyes: damage to the eyes can cause dry, red, or painful eyes. Patients may see dark floating spots and have blurred vision.
- Kidneys: damage to the kidneys can cause scleroderma renal crisis, a serious condition characterized by symptoms such as sudden headaches, fever, fatigue, vision problems, mental confusion, and difficulty breathing.
- Nervous system: damage to the nervous system can cause numbness and pain in different parts of the body
Scleroderma overlap syndromes
Patients who have scleroderma overlap syndrome may develop some features of scleroderma in combination with symptoms of other conditions such as rheumatoid arthritis, dermatomyositis, systemic lupus erythematosus, and Sjögren’s syndrome.
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