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Raynaud phenomenon (RP) is an abnormal vascular response characterized by vasospasms of the arteries and arterioles of an affected area, causing pallor, pain, and numbness (McCance & Huether, 2019). This phenomenon most commonly affects the fingers but may also include toes, ears, nipples, or the nose (Johns Hopkins Medicine, 2022). The exaggerated response results from exposure to cold temperatures or emotional stress (Johns Hopkins Medicine, 2022; Wigley, 2021). Although the exact cause is still unknown, it is believed that platelet activation and aggregation (McCance & Huether, 2019) or potentially highly sensitive receptors that control vasoconstriction may both play a role (Johns Hopkins Medicine, 2022). Raynaud disease (primary Raynauds) is considered an idiopathic disease without an associated disorder, whereas Raynaud phenomenon (secondary Raynauds) occurs secondary to a systemic disease (Johns Hopkins Medicine, 2022; McCance & Huether, 2019; Wigley, 2021). Primary RP is more common yet less severe than secondary RP (Johns Hopkins Medicine, 2022).

           RP most commonly affects females, with an age of onset between 15-30 years. Risk factors and comorbidities that may exacerbate attacks include familial linkages, smoking, migraines, chemotherapy, diabetes, cardiovascular diseases, and occupation (Johns Hopkins Medicine, 2022; McCance & Huether, 2019; Wigley, 2021). Attacks are commonly bilateral, starting at the tips of the fingers and spreading proximally up the digit (McCance & Huether, 2019). During an attack, fingers typically turn white as vasoconstriction begins, which causes ischemia. The fingers may then become cyanotic as the affected area is deprived of oxygenated blood. Finally, they become red and painful and may throb as the area is reperfused (Herrick, 2017).  As attacks become more frequent and prolonged, the skin of the fingertips thickens, and the fingernails become more brittle (McCance & Huether, 2019).

           The clinical manifestations of RP can mimic or be caused by many other diseases, so it is important to work through multiple differential diagnoses. The differential diagnosis for RP may include systemic lupus erythematosus (SLE), occlusive vascular disease, and chilblains (Wigley, 2021). SLE is a systemic rheumatic disease that may cause secondary RP; this would be diagnosed instead of RP with the presence of additional symptoms such as fever, arthralgia or arthritis, and myalgias (Wigley, 2021).  Also, a highly positive antinuclear antibody (ANA) test would indicate SLE and not RP (Herrick, 2017; McCance & Huether, 2019). Nailfold capillary studies are noninvasive and ideal for differentiating RP, although they are not always available in the primary care setting.  A rheumatologist usually performs this study, and an abnormal finding would be more indicative of an autoimmune disorder and thus secondary RP (Herrick, 2017; Wigley, 2021). 

Occlusive vascular disease encompasses atherosclerosis, emboli, and thrombosis. They may mimic RP in that if a large enough vessel is occluded, it could cause limb or digit ischemia. However, what distinguishes this from RP is that it is usually asymmetrical, involving one limb, and if it involves the hand, it is usually only one finger affected. RP usually occurs symmetrically, bilaterally, and in multiple fingers. Vascular imaging with the identification of an occlusive lesion would be definitive for diagnosing (Wigley, 2021). Chilblains is also recognized as pernio. This is a cutaneous inflammatory disease that is also triggered by cold conditions. The clinical manifestations of Chilblains is swelling and erythema affecting the hands, feet, ears, and face. These symptoms are similar to RP; what differentiates Chilblains is that it manifests as papules, patches, and nodular pruritic lesions, whereas RP does not (Wigley, 2021). 

The diagnosis of RP requires that the symptoms of other diseases are not present; this includes the absence of tissue necrosis and no other identifiable cause, which would classify the disorder as secondary RP (McCance & Huether, 2019).  A negative ANA and a normal nail capillary study would also support a diagnosis of primary RP (Herrick, 2017; Wigley, 2021). There is currently no cure for RP. Treatment consists of symptom management and avoiding triggers such as cold temperatures, emotional stress, and smoking. In severe cases, vasodilators may be prescribed to decrease the severity of vasoconstrictive attacks (McCance & Huether, 2019). If secondary RP is diagnosed, treatment of the underlying condition should help with the symptoms of RP (Johns Hopkins Medicine, 2022; Wigley, 2021). 

Have you ever encountered someone who has RP?

How was it debilitating for them, and how could you educate them to help with their debilitation?

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