Scleroderma is a rare disease of the connective tissue. It can cause the tissue in skin, joints, and internal organs to thicken and stiffen. There are three major forms of the disease:
Localized scleroderma (also known as morphea)—usually affects only the skin in isolated parts of the body. This form is less serious.
Systemic scleroderma—affects widespread areas of skin and/or internal organs, most often the lungs. Certain categories of this form of scleroderma are more serious and can be fatal.
Overlap syndrome—may involve features of scleroderma and features of other autoimmune syndromes
Overproduction of collagen and other connective tissue proteins is the main feature of scleroderma, but it is not clear what causes this overproduction. Malfunction of the immune system may contribute to excess collagen production. There is also evidence that scleroderma may result from vascular abnormalities.
These factors increase your chance of developing scleroderma. Tell your doctor if you have any of these risk factors:
Age: 30-50 years old
Occupational exposures (eg, polyvinyl chloride or silica dust)
Symptoms are usually restricted to the skin. This disorder does not progress to involve internal organs. The skin lesions may completely reverse themselves in a few months or a few years. In some cases, they lead to permanent disfigurement. Symptoms include:
Hard patches on the skin, most often on the face or trunk (morphea)
Lines of thickened skin that can extend to underlying muscles and bones (linear scleroderma or linear morphea)
This form of the disease is typically categorized as either limited or diffuse disease. Many cases of limited disease begin gradually with
. This involves swelling, tingling, numbness, blue and white color, and pain of fingers and toes. It is brought on by cold or emotional distress. The condition can progress over the years to thickened skin.
Kreuter A, Hyun J, Stücker M, Sommer A, Altmeyer P, Gambichler T. A randomized controlled study of low-dose UVA1, medium-dose UVA1, and narrowband UVB phototherapy in the treatment of localized scleroderma.
J Am Acad Dermatol
Mathai SC, Girgis RE, Fisher MR, Champion HC, Housten-Harris T. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension.
Eur Respir J
Please be aware that this information is provided to supplement the care
provided by your physician. It is neither intended nor implied to be a
substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER
IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the
advice of your physician or other qualified health provider prior to
starting any new treatment or with any questions you may have regarding a