Systemic Scleroderma

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Systemic Scleroderma (also known as Systemic Sclerosis): 

Limited cutaneous scleroderma typically comes on gradually and affects the skin only in certain areas: the fingers, hands, face, lower arms, and legs. Most people with limited disease have Raynaud’s Phenomenon for years before skin thickening starts. Telangiectasia and calcinosis often follow (see definitions below). Gastrointestinal involvement occurs commonly, and some patients have severe lung problems, even though the skin thickening remains limited.

People with limited disease often have all or some of the symptoms that some doctors call CREST, which stands for the following:

 

Calcinosis (KAL-sin-OH-sis): These are small white chalky lumps made up of calcium that form under the skin. They often occur around the fingers but can occur anywhere in the body. They are not caused by too much calcium in the diet. They can rupture through the skin causing the oozing of a chalky white material.

Raynaud’s Phenomenon (ray-NOHZ):  A condition caused by a constriction or narrowing of the blood vessels. Cold exposure or emotional stress can trigger such spasms. This leads to a cycle where the fingers first blanch (go white), then turn very blue, and then return to normal colour. Although it usually occurs in the fingers, the toes can also be involved. Smoking aggravates this condition.

Esophageal dysfunction (eh-SOFF-uh-GEE-ul):  Food is normally carried down the esophagus from the mouth to the stomach by a rhythmic muscle action that moves it along. The esophagus becomes less mobile, the muscle action diminishes, and over time, the muscle may be replaced by scar tissue, causing it to be an open pipe more than a dynamic muscular tube. This can result in difficulty swallowing. Heartburn occurs as acid from the stomach is able to flood upwards into the esophagus where it can cause burning. There may be a feeling of bloating when eating and a desire to vomit after eating.

Sclerodactyly (SKLER-oh-DAK-till-ee): This means "hardness of the digits." The skin of the fingers is dry, coarse textured or "woody" feeling. Hair vanishes, creases disappear, and the whole area looks shiny. There is accompanying difficulty in moving the fingers. The fingers tend to flex or tighten into a bent position.

Telangiectasia (tel-AN-jee-ek-TAY-zee-uh): These are small red spots that appear on the surface of the skin and are due to enlarged (damaged) small blood vessels. They commonly occur on the fingers, palms, face, lips and tongue. 

 

Bowel Dysfunction:  Replacement of the muscle, the intestine, results in changes similar to that seen in the esophagus. This can lead to the inability to absorb nutrients from food, an overgrowth of bacteria in the bowel, weight loss, and diarrhea or constipation.

This condition typically comes on suddenly. Skin thickening begins in the hands and spreads quickly and over much of the body, affecting the hands, face, upper arms, upper legs, chest, and stomach in a symmetrical fashion (for example, if one arm or one side of the trunk is affected, the other is also affected). Some people may have more area of their skin affected than others. Internally, this condition can damage key organs such as the intestines, lungs, heart, and kidneys.

People with diffuse disease are often tired, lose appetite and weight, and have joint swelling or pain. Skin changes can cause the skin to swell, appear shiny, and feel tight and itchy.  The damage of diffuse scleroderma typically occurs over a few years. After the first 3 to 5 years, people with diffuse disease often enter a stable phase lasting for varying lengths of time. During this phase, symptoms subside: joint pain eases, fatigue lessens, and appetite returns. Progressive skin thickening and organ damage decrease.  Gradually, however, the skin may begin to soften, which tends to occur in reverse order of the thickening process: the last areas thickened are the first to begin softening. Some patients’ skin returns to a somewhat normal state, while other patients are left with thin, fragile skin without hair or sweat glands. Serious new damage to the heart, lungs, or kidneys is unlikely to occur, although patients are left with whatever damage they have in specific organs. 

People with diffuse scleroderma face the most serious long-term outlook if they develop severe kidney, lung, digestive, or heart problems. Fortunately, less than one-third of patients with diffuse disease develop these severe problems. Early diagnosis and continual and careful medical monitoring are important in order to help manage symptoms and prevent health complications.

This is the term for a form of systemic sclerosis where patients do not have any overt skin thickening, but do have other manifestations of systemic sclerosis which may involve the blood vessels and major organs.

 

 

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