A 21-year-old Caucasian woman with no past medical history presents with 9 months of painful color changes of her fingers upon cold exposure. She has no other complaints, and a thorough review of systems is negative. Her family history is significant for her mother having rheumatoid arthritis. She is a nonsmoker. Physical examination is normal other than dilated capillary loops and capillary dropout on nailfold capillary microscopy. Laboratory values reveal a positive antinuclear antibody (ANA) of 1:160 but are otherwise unremarkable. She is diagnosed with Raynaud phenomenon (RP).
RP is an exaggerated, reversible, vasospastic response of acral blood vessels upon exposure to cold or emotional stress. Clinically, it is characterized by sharply demarcated color changes of the digits often accompanied by pain or paresthesias (Figure 74-1).
Raynaud phenomenon (RP). Note the sharply demarcated color changes with some digits demonstrating pallor (ischemia) and others the blue-purple discoloration of deoxygenation. (Photograph courtesy of Dr. Steven M. Dean.)
The true prevalence of RP is difficult to establish because there is no gold standard diagnostic test.
Estimated prevalence between 2% and 20% of the population.1,2,3, and 4
Female-to-male ratio between 3:1 and 9:1.1,2,3, and 4
More common in younger age groups; mean age of onset in second to third decades.
Equal occurrence across races.
ETIOLOGY AND PATHOPHYSIOLOGY
Primary RP (PRP) is the term used when there is no identifiable underlying disorder contributing to the vasospasm and there is no history of tissue damage from ischemia.
Secondary RP (SRP) occurs in the setting of an underlying disorder, most often a rheumatologic disease, and is often complicated by significant morbidity from digital ischemia (Table 74-1 and Figures 74-2,Figures 74-3, and 74-4). In severe disease with prolonged ischemic attacks, destruction of the distal phalanges may occur—a phenomenon known as acro-osteolysis (Figure 74-5).
TABLE 74-1.Secondary Raynaud Phenomenon–Associated Conditions and Factors |Favorite Table|Download (.pdf) TABLE 74-1. Secondary Raynaud Phenomenon–Associated Conditions and Factors
Systemic lupus erythematosus
Dermatomyositis or polymyositis
Mixed connective tissue disease
Undifferentiated connective tissue disease
Thoracic outlet syndrome
Thromboangiitis obliterans (Buerger disease)
|Carpal tunnel syndrome |
|Drugs or Chemicals |
Digital necrosis. Sharply demarcated necrosis of the fingertips in a patient with limited cutaneous systemic sclerosis associated with severe secondary Raynaud phenomenon (RP).