Scleroderma is a group of rare diseases that cause hardening of the skin and connective tissues. Localized scleroderma affects just the skin, whereas systemic scleroderma also affects the internal organs such as the lungs, kidneys and heart. An estimated 300,000 people in the United States have scleroderma, according to the Scleroderma Foundation, with about one-third of them suffering from the systemic form of the disease. Symptoms of the disease depend greatly on the type of scleroderma a person has.
Causes
Scleroderma occurs due to an overproduction collagen, the fibrous protein that makes up the bodies connective tissues. Physicians are not sure what sets off this overproduction of collagen, according to MayoClinic.com. They do know that the disease is triggered by the body's immune system turning on itself, causing the overproduction of collagen and producing inflammation.
Symptoms
When scleroderma affects the skin, symptoms often include hardening of the skin, hair loss, skin with light or dark patches, sores on the fingertips and toes and thickening, tightening or stiffness in the forearms, hands and fingers. People may have lumps beneath the skin that sometimes ooze a white substance with a toothpaste-like consistency, according to the National Institutes of Health. The facial skin may appear tight and mask like. People may also suffer from Reynaud's phenomenon, in which the skin on the fingers turns red, blue or blanches in response to heat or cold. People suffering from systemic scleroderma may experience joint pain, stiffness and swelling of the fingers, wrist pain, dry cough, shortness of breath, constipation, bloating after meals, diarrhea and difficulty swallowing.
Time Frame
The disease commonly affects people between the ages of 35 and 55. The University of Maryland Medical Center explains that localized scleroderma can affect children but it is very rare, occurring between 0.2 and 0.4 per 100,000 people.
Risk Factors
The University of Maryland Medical Center explains that a family history is the greatest risk factor for developing scleroderma. Women have a three to eight times higher incidence rate of developing scleroderma. People with Choctaw Native American ancestry have a high risk of developing the disease, as do African Americans.
Complications
Most of the complications of scleroderma lie in people suffering from the systemic form of the disease. Scleroderma affecting the heart or kidneys can lead to organ failure over time. When scleroderma begins in the digestive tract, it can cause malabsorption of vitamins and minerals leading to muscle wasting and weight loss. Scleroderma in the lungs can lead to pulmonary high blood pressure and pulmonary fibrosis, which can result in death.
