What Is Scleroderma?
An autoimmune disease that hardens the skin and connective tissue, and can affect internal organs. Understanding the types and the serious complications.
Scleroderma is an autoimmune disease involving hardening and tightening of the skin, connective tissues, and sometimes internal organs such as the lungs, kidneys, gastrointestinal system, and heart. It results from abnormal and excessive production of collagen, a fibrous protein.
Scleroderma affects women more often than men and most commonly occurs between ages 30 and 50. It is caused by a combination of genetic, environmental, and immune-system factors.
There are two main types. Localized scleroderma primarily affects the skin (and can involve muscles, joints, and bones) but not internal organs; the skin can become hard and thick on the arms and legs. Diffuse systemic scleroderma is more serious and can involve multiple organ systems and blood vessels.
CREST syndrome is a limited form with skin thickening confined to the fingers and toes, plus Calcinosis (calcified skin nodules), Raynaud's phenomenon, Esophageal motility problems, Sclerodactyly, and Telangiectasias (prominent skin blood vessels). A serious complication is pulmonary hypertension, elevated blood pressure in the pulmonary artery. The centromere antibody is often seen with this form.
I first saw this disease as an intern, when a young woman arrived with hard, contracted fingers, severe blood abnormalities, and kidney failure, a life-threatening scleroderma renal crisis. She nearly died, but was started immediately on the right medication and survived, a reminder of how crucial prompt recognition and treatment can be.
Dr. Maggie Cadet, MD