Pathogenesis of the Raynaud phenomenon
- Fredrick M Wigley, MD
Fredrick M Wigley, MD
- Professor of Medicine
- Johns Hopkins University School of Medicine
One of the normal physiologic responses to cold temperature is the lowering of blood flow to the skin, thereby reducing the loss of body heat and preserving normal core temperature. Blood flow to the skin is regulated by a complex interactive system involving neural signals, circulating hormones, and mediators released from both circulating cells and blood vessels.
Raynaud phenomenon (RP) is an exaggerated vascular response to cold temperature or emotional stress. The phenomenon is manifested clinically by sharply demarcated color changes of the skin of the digits. Abnormal vasoconstriction of digital arteries and cutaneous arterioles due to a local defect in normal vascular responses is thought to underlie the primary form of this disorder [1-4].
RP is considered primary if these symptoms occur alone without evidence of any associated disorder. By comparison, secondary disease refers to the presence of RP in association with a related illness, such as systemic lupus erythematosus and systemic sclerosis (SSc).
The pathogenesis of RP will be reviewed here. The definition, clinical manifestations, diagnosis, and treatment of the disorder are presented separately. (See "Clinical manifestations and diagnosis of the Raynaud phenomenon" and "Initial treatment of the Raynaud phenomenon" and "Treatment of the Raynaud phenomenon resistant to initial therapy".)
Maurice Raynaud in 1862 stated that "local asphyxia of the extremities" was a result of "increased irritability of the central parts of the cord presiding over the vascular innervation" . In 1930, after observing that even when reflex vasodilation is produced by warming the body, vasospasm could still be induced by putting the hands in cold water, and conversely, that vasospasm could not be produced by body cooling if the hands were kept warm, Sir Thomas Lewis concluded that Raynaud phenomenon (RP) was due to a "local fault" rather than a defect in the central nervous system . A local defect(s) is hypothesized to be responsible for RP. However, the exact abnormality may vary depending upon the underlying cause .
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