Wednesday, October 8, 2008 - 1:51AM EST

Introduction to Scleroderma

Raynaud's Phenomenon

One of the first symptoms of systemic scleroderma (SSc) is Raynaud's phenomenon (RP) which was first documented by Maurice Raynaud in 1862. He described symptoms whereby patients feel cold hands and feet when exposed to cold temperatures or to emotional stress. Raynaud's phenomenon is characterized initially by a paleness or whitening of the skin then cyanosis (bluishness) of the skin and finally erythema (reddening) of the skin accompanied by tingling of the fingers as the blood flow returns to the extremity. Raynaud's phenomenon can be highly variable among people, lasting from minutes to hours and ranging from being painless to sensations of tingling, numbness and throbbing. This reaction is caused by changes in the small arteries and capillaries of the hands and feet.

Raynaud's phenomenon is a nearly universal finding in people who are diagnosed with systemic scleroderma ("secondary RP"). It is rarely seen in localized scleroderma. Patients with limited systemic scleroderma may suffer from RP for several years before other symptoms appear, whereas, in patients with diffuse systemic scleroderma, the interval is much shorter (weeks or months) between the appearance of RP and progressive, rapid skin changes. Raynaud's phenomenon is considered a major criterion for the diagnosis of limited or diffuse scleroderma in both children and adults.

Approximately 5-10% of the U.S. population may have RP without any underlying medical condition. This type is known as "primary Raynaud's phenomenon". There are also "Raynaud-like" symptoms which may appear with conditions such as fibromyalgia or conditions that affect the circulatory system. It is important to distinguish between RP and other similar syndromes. One of the most reliable distinguishing factors is the presence of antinuclear antibodies (ANA) when the RP is associated with systemic scleroderma.