What Is Scleroderma?

Morphea (mor-FEE-ah) is derived from the Greek word “morfi,” meaning shape or structure.

With localized morphea, the body shows one or several patches of scleroderma. Symptoms include reddish patches of inflamed and discolored skin, usually on the chest or back, but sometimes on the face, arms, and legs.

These oval-shaped patches are limited to certain areas on the body and range in size from a half-inch to approximately 12 inches in diameter. In localized morphea, there is the absence of sweat glands and very little if any, hair growth in affected areas.

Generalized morphea is a more severe, although less common, condition than localized morphea. Large dark patches of thick, tight skin form on the trunk, arms, and legs, and can be so extensive that they merge into one another. These widespread plaques closely resemble oversized patches of localized morphea but are usually ivory-colored and sometimes have a distinct violet-colored border.

Patients with generalized morphea do not suffer from the internal life-threatening features of systemic sclerosis, but the disease can result in severe scars and physical disability. Generalized morphea has been known to appear on areas of the skin treated by radiation therapy.

In many cases, both localized and generalized morphea improve spontaneously over time (typically 3 through 5 years); however, patients are often left with patches of darkened or discolored skin and, in rare cases, muscle damage.

Linear scleroderma most commonly occurs in childhood. This type of scleroderma causes tight, thick bands to appear on the extremities, the back and front of the trunk, the buttocks, or the face. They often appear as a single band in one body area and can be seen mixed with patches of morphea. Linear scleroderma can affect the bones and muscles.

When linear scleroderma occurs on the face, it is often referred to as en coup de saber (en-KOO-de-sob), a French term for “a strike of the sword,” due to its appearance.

Systemic scleroderma (both limited and diffuse) is the most dangerous form of the disease and the primary focus of research funded by the Scleroderma Research Foundation.

Systemic scleroderma affects many parts of the body. Both the limited and diffuse types of systemic scleroderma involve injury to blood vessels, inflammation, and varying amounts of fibrosis. It can affect the skin, blood vessels, muscles, joints, the gastrointestinal tract, kidneys, lungs, and heart.

Systemic scleroderma causes widespread vascular damage. There is loss of circulation that can lead to painful skin ulcers on fingers, toes, and other extremities. Inflammation and swelling cause pain and can be chronic. Loss of flexibility in the hands and severe skin tightening can impair the use of fingers and toes. The skin of the face may also tighten and harden, especially around the mouth. This makes eating, drinking, and care of teeth difficult.

About 90% of systemic sclerosis patients have a primary symptom called Raynaud’s phenomenon. With this condition, the small blood vessels of the hands, and sometimes feet, overreact to cold or stress.

The most common type of scleroderma, limited scleroderma affects about two-thirds of systemic sclerosis patients. It’s sometimes called CREST Syndrome, because of these commonly associated physical characteristics:

Calcinosis—hard, painful calcium deposits in the skin.
Raynaud’s Phenomenon—when the feet and hands turn white, then blue, then red in response to cold or stress.
Esophageal problems—trouble swallowing or acid reflux.
Sclerodactyly—tightening of the skin on the fingers and toes.
Telangiectasias—small red spots due to dilated blood vessels on the face, lips, forearms, or fingers.

Limited (CREST) scleroderma usually progresses more slowly than diffuse scleroderma.

Chronic pain, loss of mobility, and disfigurement are possible. Treating limited scleroderma symptoms is very important. The disease can still be life-threatening. It is critical to monitor for organ damage. Serious complications can occur related to the esophagus, other parts of the gastrointestinal tract, and the lungs.

The onset of diffuse scleroderma symptoms can be rapid and severe. Early in the disease, there is usually an inflammatory phase. This progresses to fibrosis— thickening and scarring of affected parts of the body. In most cases, there is extensive skin fibrosis covering the hands, arms, feet, legs, and other parts of the body.

Fibrosis of internal organs is a very dangerous complication of diffuse scleroderma involving the kidneys, lungs, heart, and gastrointestinal tract. (The lungs are the most commonly affected.) Fibrosis in the lungs causes scarring in the part of the lungs where oxygen passes into the blood vessels. This is called interstitial lung disease (ILD). Sometimes ILD can also lead to or exist with pulmonary hypertension.

People with diffuse systemic sclerosis often suffer from fatigue, weight loss, joint swelling, tightening, and pain. Patients with the diffuse scleroderma subtype should seek early diagnosis, symptomatic and disease-modifying treatment, and frequent monitoring.

Some patients do not fit the definitions of limited or diffuse systemic sclerosis. Sine (SEEN-ay) sclerosis is a rare condition that affects a small percent of people with systemic scleroderma. Patients with sine scleroderma do not have the typical hard, thick skin that is characteristic of systemic scleroderma, but still experience an abnormal buildup of scar tissue in their internal organs.