Skip to Main Content

Key Features

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

General Considerations

  • 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

  • Toes and other acral areas (eg, nose and ears) can be affected as well as fingers

  • Classified as primary (idiopathic or Raynaud disease) or secondary

  • Primary form

    • Appears between ages 15 and 30, almost always in women

    • Tends to be mildly progressive

Demographics

  • Primary RP occurs in 2–6% of adults

Clinical Findings

Symptoms and Signs

  • Only one or two fingertips may be affected in early attacks

  • All fingers down to the distal palm may be involved as condition progresses

  • Thumbs are rarely affected

  • Well-demarcated digital pallor or cyanosis in initial phase

  • Intense rubor, throbbing, paresthesia, pain, and slight swelling during recovery phase

  • Patient usually asymptomatic between attacks

  • Sensory changes that often accompany vasomotor manifestations

    • Numbness

    • Stiffness

    • Diminished sensation

    • Aching pain

  • Primary RP

    • Symmetric involvement of the fingers of both hands

    • Spasm becomes more frequent and prolonged

    • Does not cause digital pitting, ulceration, or gangrene

  • Secondary RP

    • May be unilateral and may involve only one or two fingers

    • Nailfold capillary abnormalities

    • Digital pitting or ulceration

    • Skin tightening

    • Loss of extremity pulse

    • Rash

    • Swollen joints

Differential Diagnosis

  • RP occasionally the first manifestation of such rheumatic conditions as

    • Systemic sclerosis (including its CREST variant)

    • Systemic lupus erythematosus

    • Mixed connective tissue disease

  • Thromboangiitis obliterans (Buerger disease)

  • Thoracic outlet syndromes

  • Acrocyanosis

  • Frostbite

  • Ergot poisoning (unusual)

  • Bleomycin and vincristine chemotherapy

  • Cryoglobulinemia

  • Erythromelalgia can mimic the rubor phase of RP; exacerbation by heat and relief with cold readily distinguish erythromelalgia from RP

Diagnosis

Laboratory Tests

Table 20–7.Frequency (%) of autoantibodies in rheumatic diseases.1

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.