Scleroderma is a chronic disease that literally means "hard" (sclera) "skin" (derma). This disease affects almost 300,000 people in the United States, according to the Scleroderma Foundation. It can range from mild skin lesions to serious organ damage. Scleroderma has two main types--localized scleroderma and systemic scleroderma. Systemic scleroderma is further divided into two types--limited cutaneous scleroderma and diffuse cutaneous scleroderma. Each type of scleroderma has its own set of symptoms.
Localized Scleroderma
Localized scleroderma affects only the skin and nearby tissues but not the internal organs. There are two sub-types of localized scleroderma--morphea and linear.
The initial symptom of morphea scleroderma is the appearance of oval-shaped red patches of skin that can become thick. Over time the center of these lesions turns ivory while the edges turn violet. These lesions may remain in a small localized area or occur in other areas of the body as well. Some patients with morphea will also experience weak muscles.
Linear scleroderma occurs when a single line of skin becomes discolored or hard. It commonly occurs down an arm, a leg or even on the forehead.
Some cases of localized scleroderma will resolve over time while others are chronic and can result in debilitation.
Limited Cutaneous Scleroderma
The onset of limited cutaneous scleroderma is usually gradual and most often occurs in certain areas like the fingers, hands, face, lower arms and legs. The first symptom many patients notice is Raynaud's phenomenon, which occurs when the small blood vessels in the hands and feet narrow, resulting in the fingers and toes turning blue and feeling cold. Other symptoms of limited cutaneous scleroderma are calcinosis, esophageal dysfunction, sclerodactyly and telangiectasia.
Calcinosis is the formation of calcium deposits in the connective tissue. Although these can only be detected through X-rays, if they break away through the skin they can cause painful ulcers.
Esophageal dysfunction occurs when the smooth muscles of the esophagus become hard, which inhibits its ability to effectively move food from the mouth to the stomach.
Scleroderma causes excess collagen production, and when that collagen deposits in the layers of skin on the fingers they become thick and tight. This condition is known as sclerodactyly.
Talangiectasia is when the small blood vessels in the hands and face swell, producing a rash of small red dots.
Diffuse Cutaneous Scleroderma
Diffuse cutaneous scleroderma occurs suddenly and is often serious. As the disease begins the skin of the hands and feet thicken, but it quickly spreads throughout the body. Additional symptoms include joint pain, swollen joints, fatigue, loss of appetite and weight loss. Permanent organ damage can occur, but according to the National Institute of Arthritis Musculoskeletal and Skin Diseases only approximately one-third of patients will develop severe problems.
Diffuse cutaneous scleroderma often results in damage to organs, including the lungs, intestines, heart and kidneys.
