Raynaudís phenomenon was first described in 1862 by Dr. Raynaud, who was a French medical student at the time. He described a three-phase color change (white, blue, red) seen in the hands of women (although these changes may be seen elsewhere, including the toes and nose) when exposed to the cold.
The initial phase is that of vasoconstriction producing a white pallor of the fingers. Venous stasis ensues, producing a bluish discoloration, referred to as the cyanotic phase. This is followed by a return of color and redness, the erythematous phase, which may be accompanied by pain or paresthesias.
Similar symptoms can be brought on by immersion of the hands in cold water, or by heightened emotion or anxiety, or even going into air conditioning on a hot summer day.
Patients without detectable underlying disease or vascular abnormalities are said to have Raynaudís disease. Patients in whom Raynaudís occurs in conjunction with another underlying rheumatic disease are said to have Raynaudís syndrome.
The pathogenesis of Raynaudís phenomenon is not fully understood. Certainly, increased vascular sympathetic tone plays a role.
Raynaudís disease tends to affect females more often than males and is most often seen as an isolated finding. However, patients with a positive ANA (anti-nuclear antibody), with physical findings suggestive of abnormalities in the nailfold capillaries, or with digital ulcers, may be at a higher risk for evolution to a unifying rheumatic disease.
Raynaudís syndrome may accompany a number of rheumatic diseases including (but not limited to) systemic lupus erythematosus (SLE) and scleroderma. While many of these children will not develop rheumatic disease over time, all children with Raynaudís symptoms deserve a comprehensive history, physical exam, appropriate laboratory testing, and a careful rheumatology evaluation.
If you have a patient with Raynaudís symptoms, please contact HSS Pediatrics and we will be happy to help.