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11/19/2018

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P.O. Box 827
Shingle Springs, CA 95682
Tel: (530) 672-2473


What is Scleroderma?

A Broad Overview of the Disease

Hard (Skleros) – Skin (Derma)


Scleroderma is an autoimmune connective tissue disorder that is very diverse in how it affects an individual. This diversity along with the relative obscurity of the disease can make it difficult to diagnose. It is not uncommon for a patient to go undiagnosed for a significant period of time, including years.

It is estimated by several sources that about 300,000 Americans suffer from this debilitating disease of which 80% are female. Of these women, it typically strikes them between the ages of 25 and 55.

Scleroderma in the simplest of terms is an overproduction of collagen and falls into the same category as multiple sclerosis, lupus and rheumatoid arthritis. The production of collagen in a healthy person promotes the softness of the skin. Ironically, the overproduction causes soft tissue to become hard.

Although it is not life threatening in the localized version, systemically the disease is much more challenging and debiltating.


Symptoms


Some of the symptoms that are typical of Scleroderma can include but are not limited to:

  • Body/Muscle Pain
  • Discoloration of Skin
  • Raynaud’s Phenomenon
  • Thickening of Skin
  • Difficulty Swallowing
  • Joint Pain and Stiffness
  • Fatigue
  • Internal Organ Damage
  • Dry Mucous Membranes
  • Weight Loss


Types of Scleroderma

There are two types of Scleroderma that also have subsets, localized and systemic.


Localized Scleroderma


Morphea
This form of the disease typically only involves the skin. The face, trunk and extremities can have thick tight skin or may be affected by oval patches of inflamed skin. It can be disfiguring and very painful.

Linear
Bands of skin become thick tight and can form around the truck arms and legs. In some case it may extend down through tissue to the bone, causing associated loss of deep tissue or bone structure.


Systemic Scleroderma


Raynaud’s phenomenon is a primary symptom of both limited and diffuse systemic scleroderma. In scleroderma patients, Raynaud’s phenomenon is accompanied by vasospasm of the small blood vessels in the extremities, which causes, first, the constriction and, then the return of blood flow—a mechanism that injures and eventually destroys some of the small vessels.

In both forms of systemic scleroderma, loss of circulation can result in painful skin ulcers that occur on fingers, toes, and other extremities. Joint pain caused by inflammation and swelling can be chronic. Joint contractures and loss of flexibility in the hands resulting from skin swelling and tightening may develop. The skin around the face may tighten and harden, especially around the mouth.

Limited 
Also know as CREST, is a milder form of the disease than systemic-diffuse. Crest is an acronym for several of its prominent symptoms:

Calcinosis - Painful calcium deposits in the skin
Raynauds Phenomenon – Sensitivity to cold in fingers and hands
Esophageal Dysfunction – Problems with swallowing caused by internal scarring
Sclerodactyly - Tightening of the skin on the fingers, toes and hands.
Telangiectasia – Red spots on the hands, palms, forearms, face and lips.

Organ involvement is late into the disease and has a slower progression. Skin thickening is limited to sites distal to the elbow and knee but also involving the face and neck.


Diffuse
This form places the individual at the most risk of serious internal involvement. Unlike limited scleroderma, the onset of diffuse symptoms can be rapid and severe. Typical symptoms of this form are extensive skin involvement (fibrosis) as well as lung, heart, gastrointestinal or kidney. Whole body pain, disfigurement, serious disability and organ failure are all possible with systemic-diffuse scleroderma.


Sine
This form of the disease also strikes the internal organs but without the characteristic skin involvement. Consequently, it can be very difficult to diagnose.


Overlap
Scleroderma can also occur in patients with other autoimmune disorders as part of an overlap.

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