Scleroderma: A Rare but Devastating Disease

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As the disease progresses, the skin grows thicker. "It can affect your movement," Merkel said. "It can affect your ability to move your hands in a functional way. The skin on your face becomes very tight. It can change people's appearance."

The thickening can also spread, becoming systemic. The fingers, hands, face, lower arms and legs are the only places affected if the spread is limited, but people also can suffer a range of symptoms doctors refer to as CREST, according to the National Institutes of Health.

CREST stands for:

• Calcinosis, the formation of calcium deposits in the connective tissues, usually found on the fingers, hands, face, and trunk, and on the skin above the elbows and knees. Painful ulcers can result if the deposits break through the skin.
• Raynaud's phenomenon, a condition in which the small blood vessels of the hands or feet contract in response to cold or anxiety. Fingertips may suffer damage that leads to ulcers, scars, or gangrene.
• Esophageal dysfunction, which occurs when smooth muscles in the esophagus lose normal movement. The result can be swallowing difficulties, chronic heartburn or inflammation.
• Sclerodactyly, or thick and tight skin on the fingers resulting from deposits of excess collagen within skin layers. It becomes hard to bend or straighten fingers, and the skin may also appear shiny and darkened.
• Telangiectasia, a condition caused by the swelling of tiny blood vessels. Small red spots appear on the hands and face.

In the most serious cases, internal organs are affected. Kidneys quit working. Lungs stiffen, making it hard to take a breath. Hearts are unable to pump as efficiently.

In general, doctors treat scleroderma by treating the complications it causes, Feghali-Bostick said.

"People with lung problems are going to be treated for lung problems," she said. "People with kidney problems will be treated with drugs to prevent kidney failure."

One of the success stories in scleroderma treatment involves new autoimmune drugs that preserve kidney function. People with scleroderma used to die mainly from kidney disease, Merkel said, but these days they are more likely to die of lung problems.