Learn about Scleroderma with Project Sclerderma

LEARN ABOUT SCLERODERMA

WHAT IS SCLERODERMA?

Source: John Hopkins Scleroderma Center.
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Scleroderma is classified as an autoimmune disease. This means that a person’s immune system works against itself. The normal immune system protects the body by fighting off foreign invaders such as viruses and bacteria. In an autoimmune disease, the immune system mistakes a person’s own tissues as foreign invaders and attacks the wrong things.
In scleroderma, cells start making collagen as if there were an injury that needs repairing. The cells do not turn off as they should and end up making too much collagen. The extra collagen in the tissues can prevent the body’s organs from functioning normally. This is what is often referred to as fibrosis or “scar tissue”.
The classification of scleroderma by skin manifestations:

LIMITED SCLERODERMA

Limited scleroderma means only limited areas of skin are thick; usually just the fingers and/or face. Limited scleroderma is the milder form of scleroderma. It is more common among Caucasians than other populations. Every person with scleroderma is different and has a different pattern of symptoms.  The CREST syndrome is a type of limited scleroderma. 

Calcium in the skin and tissues can be a painful annoyance that can irritate or break the skin surface. Raynaud’s attacks can be uncomfortable and associated with cold intolerance. Acid reflux from esophageal dysmotility can be painful causing irritation in the lining of the esophagus. The skin changes in limited scleroderma usually do not progress. Generally, limited scleroderma causes less involvement of body organs than the more severe form.

CREST Syndrome

C

(calcinosis) is for the calcium deposits under the skin and in tissues.

R

stands for Raynaud’s phenomenon.

E

is for esophageal dysmotility. This causes heartburn, which is often experienced by CREST patients.

S

is for sclerodactyly; that means thick skin on the fingers.

T

is for telangiectasias, which are enlarged blood vessels. These appear as red spots on the face and other areas.

C

(calcinosis) is for the calcium deposits under the skin and in tissues.

R

stands for Raynaud’s phenomenon.

E

is for esophageal dysmotility. This causes heartburn, which is often experienced by CREST patients.

S

is for sclerodactyly; that means thick skin on the fingers.

T

is for telangiectasias, which are enlarged blood vessels. These appear as red spots on the face and other areas.

DIFFUSE SCLERODERMA

A second type of scleroderma is called diffuse scleroderma. This means that more areas of the skin are involved and thickened, but there is a high degree of variability among patients. Skin of the arms, legs, and trunk are more likely to be involved. The tightened skin makes it difficult to bend fingers, hands, and other joints. There is sometimes inflammation of the joints, tendons and muscles.
Tight skin on the face can reduce the size of a person’s mouth and make good dental care very important. The skin can lose or gain pigment; making areas of light or dark skin. Some people lose hair on the limbs, sweat less, and develop dry skin because of skin damage. Diffuse scleroderma can have associated involvement of internal organs such as the gastrointestinal tract, heart, lungs, or kidneys. The degree of organ involvement is highly variable – some get none at all and other patients organs may be badly affected.
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WHAT ARE THE SYMPTOMS?

Among the early signs of scleroderma is Raynaud’s phenomenon (pronounced RAY-KNOWDS), which is identified by fingers that become very sensitive to cold, causing the fingers to change colors. The changes of color are caused by the spasm and narrowing of the blood vessels. This reaction can be caused by emotional stress as well. Additionally, fingers and hands that become stiff and puffy can also be early indicators of Scleroderma. There are two types of Raynaud’s Phenomenon:

PRIMARY RAYNAUD'S

Raynaud’s phenomenon is a common condition and most people with Raynaud’s phenomenon will NOT develop scleroderma. These individuals have what is referred to as primary Raynaud’s.

SECONDARY RAYNAUD'S

While only a small percentage of people with Raynaud’s phenomenon develop scleroderma, almost all scleroderma patients have Raynaud’s symptoms. This is referred to as secondary Raynaud’s phenomenon, so-named because it is secondary to the scleroderma.

Scleroderma patient support through education and spreading an understanding of the causes

WHAT CAUSES SCLERODERMA?

At the moment there is no known cause of scleroderma and no way of preventing this disease. It is more common in females than males, yet it affects all genders, all ages and all racial groups. The peak of the disease onset occurs in the fourth and fifth decades of life. Familial scleroderma is rare, and there is no evidence of genetic inheritance.

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