Scleroderma and systemic sclerosis

Scleroderma and Systemic sclerosis

Scleroderma is an autoimmune disease. In this case it means that the immune system attacks the connective tissue. This causes skin thickening, spontaneous scarring, blood vessel disease, and several degrees of inflammation. Scleroderma patients have fibrosis in the skin (scar tissue) and this leads to thickness and firmness of the involved skin. This condition can also appear in internal organs.

Scleroderma is classified depending on the degree and location of the skin and organ involvement:

- localized scleroderma: This is the mildest form of scleroderma. It often affects children, but can occur at any age. It is characterized by skin changes, which can be in isolated areas either as „morphea patches“ (localized in a patchy area of the skin that becomes hard and slightly pigmented and it can cause several lesions in the skin) or as „linear scleroderma“ (it is usually located in a lower extremity and it frequently looks like a strip of hardening skin down the leg). Linear scleroderma can happen in children and it can stunt bone growth of the affected limb. Sometimes linear scleroderma is associated with a „satellite“ area of a patch of localized scleroderma skin, such as on the abdomen.

- Widespread scleroderma or systemic scleroderma: This type mostly affects women and usually develops between 30 and 50 years of age. Children are rarely affected. It has a tendency to affect skin and also internal organs. Systemic scleroderma is further divided into diffuse and limited, depending on the extent of skin involvement. Diffuse scleroderma is also called diffuse systemic sclerosis and it happens when the skin becomes thick in the extremities, face and trunk (chest, back, abdomen, or flanks). This condition can rapidly cause inflammation and skin hardening. The disease affects the organs such as esophagus, bowels, and scarring (fibrosis) of the lungs, heart and kidneys and if not treated on time it often leads to death. If the patient has high blood pressure, this can lead to renal crisis (kidney failure).

The limited scleroderma is also called CREST scleroderma because it involves less skin thickening in fingers, hands and face and because skin changes and other features of the disease tend to occur more slowly than in the diffuse scleroderma. The features of CREST scleroderma include: Calcinosis, Raynaud's phenomenon, Esophagus disease, Sclerodactyly and Telangiectasias. Patients may present one, two, three or four of the following features:

Calcinosis: Occurs when tiny deposits of calcium get formed in the skin. They look like hard white areas in the superficial skin of elbows knees or fingers and can be tender, become infected and can either fall down by themselves or require an operation to remove them.

Raynaud's phenomenon: Occurs when spasm of the tiny arterial vessels appears causing the finger, toes, nose, tongue or ears to turn white, blue and red after exposure to extreme cold or even to extreme heat or emotional upset. As a consequence the finger tips develop digital ulcers (tiny areas in the finger tips become damaged) or larger areas of dead skin.

Esophaugus disease: Occurs when the muscle of the lower two-thirds of the esophagus works poorly. As a consequence the esophagus becomes wider and allows stomach acid to backflow into the esophagus to cause heartburn, inflammation, and potentially scarring that narrows the esophagus. This situation makes also difficult to swallow food through the esophagus into the stomach and patients with scleroderma need to be treated specially to protect the esophagus from heartburn.

Sclerodactyly: Occurs when the skin of the fingers or toes becomes thick and tight causing a shiny and slightly puffy appearance. The tightness can cause severe limitation of motion and the skin changes generally progress much slower than those of people with diffuse form of scleroderma.

Telangiectasia: Is characterized by tiny red areas generally on the face, hands and mouth (behind the lips), which blanch when they are pressed upon (widened capillaries).

It is important to mention that few patients can present CREST features but then it turns into the diffuse form of scleroderma. Further, some patients present overlaps of scleroderma and other diseases such as rheumatoid arthritis, systemic lupus erythematosus and polymyositis. A condition called mixed connective tissue disease (MCTD) can also occur when the patient presents scleroderma features together with features of polymyositis, systemic lupus erythematosus and certain abnormal blood tests.

Please call your doctor if you think you might have this condition.