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 can also affect internal organs such as the heart, lungs, kidneys, and intestinal tract.
Symptoms can vary dramatically, depending on which organs are affected and how severely. For some patients, symptoms can be mild; for others, they can be life-threatening.
Localized versus systemic scleroderma
The two major categories of scleroderma are localized and systemic.
Localized scleroderma (also known as morphea) primarily affects the skin, rarely damages internal organs, and runs a milder course. Localized scleroderma, the most common type in children, can be further classified into plaque morphea, bullous morphea, deep morphea, generalized morphea, or linear scleroderma — which may affect different areas of the body and have different clinical presentations.
Systemic scleroderma is the more serious of the two categories. Patients may experience a broad range of symptoms due to damage to internal organs.
Based on the extent of skin involvement, systemic scleroderma is further categorized into limited and diffuse, with the diffuse subtype being more severe. The subtype also affects the speed at which skin thickening develops.
The name scleroderma means hard skin in Greek. Nearly all patients with scleroderma have patches or streaks of thick and hardened skin. The skin may look shiny; affected areas also may be darker or lighter than normal and lose hair.
The hardened skin often feels tight and it may restrict movement. Skin may also feel dry or itchy. Contractures, when hardening of skin and other tissue impairs normal movement, can develop if the skin hardens over joints.
Some people with scleroderma will develop skin ulcers (sores), which are particularly common on the fingertips and toes. Calcinosis, when lumps of calcium form under the skin, can also occur.
Another common skin symptom is telangiectasias, when small blood vessels near the surface of the skin become wider than normal. These look like fine pink or red spots or lines, which briefly turn white when pressed upon.
Other skin problems commonly linked to scleroderma include sclerodactyly (tight skin over fingers that causes them to curl inward) and microstomia — a smaller mouth opening due to tight skin around the mouth, causing difficulties in eating, brushing, flossing, and speaking.
Involvement of other body systems
Almost all people with scleroderma experience Raynaud’s phenomenon. In this condition, the fingers and toes feel numb, prickly, or frigid in response to cold temperatures or emotional distress. Fingers and toes may also become abnormally pale or bluish in color. Raynaud’s phenomenon develops in people with scleroderma because of scar tissue that narrows blood vessels in the fingers and toes. In rare severe cases, Raynaud’s phenomenon can cause skin sores or gangrene (when body tissue dies due to lack of blood flow).
Different parts of the body can be affected in some cases of scleroderma, and this can result in a range of other symptoms. Some of these are:
- Muscles, bones, and joints: damage to muscle and bone tissue can cause pain and muscle weakness. Joints may become difficult or painful to move.
- 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 (when the heart cannot adequately pump blood to the body’s tissues), such as shortness of breath or heart palpitations.
- 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 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, fatigue, vision problems, mental confusion, and difficulty breathing.
- Nervous system: damage to the nervous system can cause seizures and headaches in some cases.
- Mental health: scleroderma patients may be at increased risk of certain mental health problems, such as anxiety and depression.
Scleroderma overlap syndromes
People who have scleroderma overlap syndromes develop some features of scleroderma in combination with symptoms of other conditions such as rheumatoid arthritis, dermatomyositis, systemic lupus erythematosus, and Sjögren’s syndrome.
Last updated: May 4, 2021
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