WHAT IS SCLERODERMA?
.Scleroderma literally means hard skin.
.It is a connective tissue disease affecting the skin and, in some cases, the internal organs.
.Scleroderma is classified as an autoimmune disease along with lupus, Sjogren's Syndrome, rheumatoid arthritis, and multiple sclerosis. In these conditions the immune system attacks the body's own tissues.
.A disease which varies greatly in severity from very mild to life-threatening and for most, a disease that affects the way individuals lead their daily lives.

TYPES OF SCLERODERMA
There are two basic types of scleroderma:
1. LOCALIZED form that affects the skin -- morphea and linear.
2. SYSTEMIC form that affects the internal organs -- limited or CREST and diffuse.

LOCALIZED SCLERODERMA affects the collagen producing cells in some of the areas of the skin and usually spares the internal organs and blood vessels.
Morphea occurs as patches of thick and discolored skin.
Linear is a line of thickened skin extending down an arm or leg. If it involves the forehead it is called "en coup de sabre" or "cut of the sabre". As a general rule, children tend to get the localized form and adults the systemic form.

In SYSTEMIC SCLERODERMA the immune system causes damage to two main body areas: the small blood vessels and the collagen-producing cells located in the skin and throughout the body. Narrowing of the blood vessels in the hands and feet decreases the blood supply and causes scleroderma patients to be extremely sensitive to cold. This is known as Raynaud's phenomenon and happens to about 90% of persons with systemic scleroderma. Fingers may become contracted and function is lost.
Over production of collagen, the main protein in the body's tissues, is responsible for the thick and tight skin, for the lung, kidney, gastrointestinal and heart problems is this disease. Not everyone with scleroderma will have multiple organ involvement. All combinations are seen and few patients are alike.
CREST or limited scleroderma tends to have less severe organ problems than the diffuse form. Persons with CREST have calcinosis, Raynaud's, esophagitis, sclerodactyly,and telangectasias. SEE column right.

OVERLAP
Scleroderma can also be found in persons with other autoimmune diseases such as lupus, rheumatoid arthritis, Sjorgren's syndrome or polymyositis, as part of an overlap syndrome or a mixed connective tissue disease.

ENVIRONMENTAL
A variety of drugs and chemicals in the environment can produce lesions that are similar to those seen in scleroderma.

TYPES OF SCLERODERMA

1. Localized scleroderma
(Not involving internal body systems)
*Morphea
*Linear

2. Systemic Scleroderma
(Involving internal systems)
*Limited or CREST
*Diffuse

3. Scleroderma-related disorders
*Overlaps with other autoimmune diseases
*Environmental scleroderma-like diseases

C R E S T

CALCINOSIS - refers to calcium deposits in the skin
RAYNAUD'S PHENOMENON - color changes in fingers with exposure to cold. May affect feet and nose
ESOPHAGEAL DYSFUNCTION - acid in the espohagus felt as heartburn
SCLERODACTYLY - tight and thick skin of the fingers
TELANGIECTASIAS - a particular type of red spots on the skin

WHO GETS SCLERODERMA?

.Female patients outnumber male patients about four to one, and the average age at diagnosis is in the forties. However, scleroderma can occur in all age groups and variations frequently occur. Each case is different. In fact, exception seems to be the rule in scleroderma.

WHAT CAUSES SCLERODERMA?

.There is no known cause or cure as yet.
.It is not hereditary but research indicates that there is a susceptibility gene which raises the likelihood of getting scleroderma but by itself does not cause the disease.