Description

• Idiopathic intermittent episodes of vasoconstriction of digital arteries, precapillary arterioles, and cutaneous arteriovenous shunts in response to cold temperatures or emotional stress:
  • A triphasic color change of the fingers (occasionally the toes, rarely nipples) is the physical manifestation of the episodes:
    • Thumbs are rarely involved.
    • The initial color is white from extreme pallor, then blue from cyanosis, and finally with warming and vasodilatation intense redness develops.
  • Swelling, throbbing, and paresthesias are the final symptoms.
  • Primary:
    • 80% of patients with Raynaud phenomenon have primary disease.
    • Episodes are bilateral and nonprogressive.
    • Diagnosis confirmed only if after ≥2 years of symptoms, no underlying connective tissue disease develops
  • Secondary:
    • Progressive and asymmetric
    • Spasm is more frequent and more severe with time. No gangrene; rarely, ulceration; 13% may progress to atrophy of digital fat pads and ischemic ulcers of fingertips.
    • Usually associated with an underlying connective tissue disease
• System(s) affected: Hematologic; Lymphatic; Immunologic; Musculoskeletal; Dermatologic; Exocrine

• Raynaud phenomenon can appear as breast pain in lactating women (1).
• Bacterial culture of breast milk can distinguish mastitis from Raynaud phenomenon.

Geriatric Considerations
Initial appearance of Raynaud phenomenon at >40 frequently indicates an underlying connective tissue disease.

Pediatric Considerations
Associated with systemic lupus erythematosus (SLE) and scleroderma

Epidemiology

• Primary:
  • Predominant age: 14 years; ~1/4 begin >40
  • Predominant sex: Female > Male (4:1)
• Secondary:
  • Predominant age: >40
  • Predominant sex: No gender predilection

• Primary: ~3–12.5% of men; 6–20% of women (based on reporting of characteristic skin color changes, intolerance to cold)
• Secondary: Less common, only ~1% of population

Risk Factors

• Smoking may reduce digital blood flow but is not associated with increased risk of Raynaud phenomenon.
• Existing autoimmune or connective tissue disorder
• End-stage renal disease with hemodialysis may increase the risk due to a steal phenomenon complicating the arterial-venous shunt.
• An elevated homocysteine level has been associated with primary and secondary disease.

Genetics
Little information is available, but some studies suggest a dominant inheritance pattern. ~1/4 of primary patients have a 1st-degree relative with Raynaud phenomenon.

General Prevention

• Avoid exposure to cold.
• Stop smoking.
• Avoid trauma and vibration to hands and fingertips; however, no relationship has been established between etiology of Raynaud phenomenon and occupational vibratory tool use.

Etiology

Unknown. Functional blood vessel wall abnormalities may cause primary and structural abnormalities secondary. Serotonin receptors (5-HT₂ type) may be involved in secondary Raynaud phenomenon. Dysregulation of control mechanisms of vascular motility leads to imbalance between vasodilation and vasoconstriction. Platelet and blood viscosity abnormalities are implicated in secondary.

Commonly Associated Conditions

Secondary Raynaud:

• Scleroderma
• SLE
• Polymyositis
• Sjögren syndrome
• Occlusive vascular disease
• Cryoglobulinemia