Pediatric Diseases and ConditionsScleroderma in Children
Topic Index - Pediatric Arthritis and Other Rheumatic Diseases
Scleroderma can be either a localized disease or a disease that affects the whole body. When it affects your whole body it is also called systemic sclerosis or systemic scleroderma. Scleroderma is a chronic, degenerative disease causing abnormal growth of the connective tissue that affects the joints, skin, and internal organs. Scleroderma is also associated with blood vessel abnormalities.
Scleroderma is thought to be an autoimmune disease, meaning that the body's immune system turns on itself. Although genes play a role in the disease, it is not passed on from parents to children. In addition, unknown environmental factors likely play a role.
Scleroderma can lead to scarring of the skin, joints, and other internal organs. The following are the most common symptoms of scleroderma. However, each individual may experience symptoms differently. Symptoms may include:
Thickening and swelling of the tips of the fingers
Pale and tingly fingers that may become numb when exposed to cold or when emotionally upset (called Raynaud's phenomenon)
Taut, shiny, darker skin on large areas such as the face, that may hinder movement
Appearance of spider veins
Calcium bumps on the fingers or other bony areas
Grating noise as inflamed tissues move
Frozen (immobile) fingers, wrists, or elbows due to scarring of the skin
Sores on fingertips and knuckles
Scarring of the esophagus, leading to heartburn and difficulty swallowing
Scarring of the lungs, leading to shortness of breath
Heart failure and abnormal heart rhythms
The symptoms of scleroderma may resemble other medical conditions or problems. Always consult your doctor for a diagnosis.
In addition to a complete medical history and physical examination, a diagnosis of scleroderma is usually based on the changes in the skin and internal organs. An antibody test may help distinguish the type of scleroderma present.
Specific treatment for scleroderma will be determined by your doctor based on:
Your age, overall health, and medical history
Extent of the condition
Your tolerance for specific medications, procedures, and therapies
Expectation for the course of the disease
Your opinion or preference
Treatment may include:
Nonsteroidal anti-inflammatory medications or corticosteroids (to relieve pain)
Immunosuppressive medications, such as penicillamine (to slow the skin thickening process and delay damage to internal organs)
Treating specific symptoms, such as heartburn and Raynaud's phenomenon
Physical therapy and exercise (to maintain muscle strength)