ESSENTIALS OF DIAGNOSIS
Paroxysmal bilateral digital pallor and cyanosis followed by rubor.
Precipitated by cold or emotional stress; relieved by warmth.
Primary form: benign course; usually affects young women.
Secondary form: can cause digital ulceration or gangrene.
Raynaud phenomenon (RP) is a syndrome of paroxysmal digital ischemia, most commonly caused by an exaggerated response of digital arterioles to cold or emotional stress. The initial phase of RP, mediated by excessive vasoconstriction, consists of well-demarcated digital pallor or cyanosis; the subsequent (recovery) phase of RP, caused by vasodilation, leads to intense hyperemia and rubor. Although RP chiefly affects fingers, it can also affect toes and other acral areas such as the nose and ears. RP is classified as primary (idiopathic or Raynaud disease) or secondary. Nearly one-third of the population reports being “sensitive to the cold” but does not experience the paroxysms of digital pallor, cyanosis, and erythema characteristic of RP. Primary RP occurs in 2–6% of adults, is especially common in young women, and poses more of a nuisance than a threat to good health. In contrast, secondary RP is less common, is chiefly associated with rheumatic diseases (especially scleroderma), and can be severe enough to cause digital ulceration (eFigure 20–14) or gangrene.
Ulceration from infarction of the tip of the third digit of the left hand resulting from Raynaud phenomenon in a patient with scleroderma. (Used, with permission, from Nicole Richman, MD.)
In early attacks of RP, only one or two fingertips may be affected; as it progresses, all fingers down to the distal palm may be involved (eFigure 20–15). The thumbs are rarely affected. During recovery there may be intense rubor, throbbing, paresthesia, pain, and slight swelling. Attacks usually terminate spontaneously or upon returning to a warm room or putting the extremity in warm water. The patient is usually asymptomatic between attacks. Sensory changes that often accompany vasomotor manifestations include numbness, tingling, diminished sensation, and aching pain.
Raynaud phenomenon of the hands characterized by paroxysmal bilateral digital pallor and cyanosis followed by rubor; early in attacks, only one or two fingertips may be affected but as it progresses, all fingers down to the distal palm may be involved. (Used, with permission, from Nicole Richman, MD.)
Primary RP appears first between ages 15 and 30, almost always in women. It tends to be mildly progressive and, unlike secondary RP (which may be unilateral and may involve only one or two fingers), symmetric involvement of the fingers of both hands is the rule. Spasm becomes more frequent and prolonged. Unlike secondary RP, primary RP does not cause digital pitting, ulceration, or gangrene.