Historical Perspective of Raynaud’s Phenomenon

Abstract

Cold-induced vasospastic attacks of the fingers have been described since the eighteenth century. In 1862, Maurice Raynaud reported in his medical school graduation thesis a group of patients with spontaneous gangrene of the extremities and a history of vasospastic attacks. In such patients, fingers or toes turned white upon cold exposure or emotional stress, then cyanotic, and finally hyperemic and red when circulation was restored. Raynaud’s work was translated into English in 1888 and Raynaud’s phenomenon progressively became a worldwide used term to describe vasospastic attacks. In 1932, Allen and Brown defined the first set of classification criteria to differentiate primary Raynaud’s disease from secondary Raynaud’s phenomenon.

The mechanism of Raynaud’s phenomenon has been a 150-year-long enigma. Raynaud proposed that the vasospasm was caused by an overreactivity of the sympathetic nervous system. In 1929, Sir Thomas Lewis challenged such hypothesis showing that a “local fault” of the digital artery was actually responsible. Evidence supporting Raynaud’s or Lewis’ hypotheses alternated during the twentieth century, until the discovery of the molecular basis of cold-induced cutaneous arterial vasoconstriction led over the past decade to a unifying pathogenetic vision reconciling the two theories. Importantly, the deeper understanding of the mechanisms of Raynaud’s phenomenon has allowed the introduction of more effective targeted pharmacological therapies that have profoundly changed the current management of this disease.