Part I: Epidemiology, pathophysiology, and clinical considerations of primary and secondary Raynaud's phenomenon

Raynaud's phenomenon (RP) is a relatively common disease with both primary and secondary forms. It is well understood as a vasospastic condition affecting the acral and digital arteries, resulting in characteristic, well-demarcated color changes typically in the hands and feet in response to co...

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Bibliographic Details
Published in:Journal of the American Academy of Dermatology 2024-02, Vol.90 (2), p.223-234
Main Authors: Curtiss, Paul, Svigos, Katerina, Schwager, Zachary, Lo Sicco, Kristen, Franks, Andrew G.
Format: Article
Language:English
Subjects:
connective tissue disease
Connective Tissue Diseases - complications
Connective Tissue Diseases - diagnosis
Connective Tissue Diseases - epidemiology
erythematosus
Hand
Humans
medical dermatology
Quality of Life
Raynaud Disease - diagnosis
Raynaud Disease - epidemiology
Raynaud's phenomenon
scleroderma
systemic lupus
Upper Extremity
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Summary:Raynaud's phenomenon (RP) is a relatively common disease with both primary and secondary forms. It is well understood as a vasospastic condition affecting the acral and digital arteries, resulting in characteristic, well-demarcated color changes typically in the hands and feet in response to cold or stress. Secondary RP (SRP) has been described in association with a variety of rheumatologic and nonrheumatologic diseases, environmental exposures, and/or medications. While both primary RP and SRP may impact the quality of life, SRP may lead to permanent and potentially devastating tissue destruction when undiagnosed and untreated. It is therefore crucial for dermatologists to distinguish between primary and secondary disease forms early in clinical evaluation, investigate potential underlying causes, and risk stratify SRP patients for the development of associated autoimmune connective tissue disease. The epidemiology, pathogenesis, and clinical presentation and diagnosis of both forms of RP are described in detail in this review article.
ISSN:0190-9622
1097-6787
DOI:10.1016/j.jaad.2022.06.1199