Raynaud Phenomenon

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: more severe, sometimes causing 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: idiopathic or Raynaud disease

• Secondary form

  • Less common than primary form

  • Chiefly associated with rheumatic diseases (especially systemic sclerosis)

  • Can be severe enough to cause digital ulceration or gangrene

Demographics

• Primary RP occurs in 2–6% of adults

• Most commonly appears in women between ages 15 and 30

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

  • Tends to be mildly progressive

• 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

• Serologic tests for rheumatic diseases (Table 20–7)