Linear scleroderma is a disease characterized by hardening of the skin on the face or localized to a single extremity of the body (Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications, Thilo Gambichler, Alexander Kreuter, Klaus Hoffmann, Falk G Bechara, Peter Altmeyer, and Thomas Jansen, Department of Dermatology, Ruhr-University Bochum, Gudrunstrasse 56, D-44791, Bochum, Germany, National Institutes of Health). The disease can also invade muscle and bone. In children and young adults, linear scleroderma can interfere with proper growth in its more severe cases. In fact, this form of scleroderma generally strikes children within the first 10 years of life, and is considered a pediatric disease.
Linear scleroderma is a rheumatologic disorder because it affects connective tissues. It is a rare disease that damages the cells lining small arteries and produces an abnormal buildup of fibrosis (scar tissue) in the skin. It can cause minor to major disfiguration, but it is rarely life threatening.
Signs and Symptoms of Linear Scleroderma
Linear scleroderma, like other forms of scleroderma, causes pain, which can go from subtle to extremely debilitating pain. It also causes inflammation of the area affected. Other signs and symptoms may include, but are not limited to:
- Bone pain
- Joint pain
- Stiffness of hands and feet
- Skin discoloration
- Skin tightness
- Raynaud’s phenomenon
- Dry mucus membranes
Since linear scleroderma has no cure, the course of treatment is based on treating the symptoms that the disease causes. This is in order to make a patient more comfortable.
Diagnosis of Linear Scleroderma
Linear scleroderma is mainly diagnosed by retrieving personal history from the patient, as well as a thorough physical examination. The outward affects of linear scleroderma are easily identified by a trained eye. Blood tests to check the status of certain autoantibodies are used to help confirm a diagnosis, but are not in themselves conclusive evidence of the disease. Likewise, a doctor may order x-rays to visualize the progression of the disease, but do not use results as the sole basis for a diagnosis.
Raynaud’s phenomenon is present in almost all patients suffering from linear scleroderma. This is a condition where blood vessels are being constricted causing hands and feet to become discolored and often times painful. Having Raynaud’s phenomenon does not necessarily mean a patient has linear scleroderma, but in the presence of other symptoms and physical findings, it raises the possibility.
Treatment of Linear Scleroderma
Linear scleroderma is not a curable disease. As mentioned above, many of the treatment options are aimed at controlling the symptoms that the disease causes. For instance, keeping hands and feet warm can help with Raynaud’s, as well as vasodilator drugs. Avoiding injury to the fingers and toes can also be beneficial to a patient with Raynaud’s phenomenon.
Other measures such as limiting calcium intake can prove beneficial. Corticosteroids are often used as well to control the amount of inflammation that linear scleroderma causes (Treatment for Linear Scleroderma, Robert I. Fox, M.D., Ph.D., Allergy & Rheumatology Clinic, La Jolla, California). It is important to point out that most treatment options do not work over a consistent period of time, but only offer temporary results. Some patients do not respond to any treatment, and are monitored closely at all times.