Publish date: Posted on
Last updated: October 21, 2023
Keywords #
raynauds
raynaud phenomenon
digital ulceration
systemic sclerosis
Diagnosis #
Raynaud phenomenon (RP) is characterized by abnormal reversible vasoconstriction in response to cold temperatures and/or emotional stress, most often in the fingers and toes.[1,2,3] Rarely, the nose, ears and nipples can also be affected.[1,2] It is divided into primary Raynaud phenomenon, which is most commonly idiopathic but can also occur in individuals with atherosclerosis or those who have worked with vibrating equipment, and secondary Raynaud phenomenon, which occurs in association with underlying rheumatologic diseases.[1] It affects women more commonly than men, and most patients present in their late teens to early 30’s.[3]
RP manifests most commonly as a change in the color of the fingers and toes, classically seen as a progressive change from normal skin color to white, then from white to blue, and finally from blue to red, although it does not have to follow this specific pattern.[1] RP is a vasospastic disorder, in which cold temperature or emotional stress causes abnormal vasoconstriction, which leads to less blood reaching the fingers and toes resulting first in pallor of the affected regions. Less blood to the tissues leads to local hypoxemia which is seen as cyanosis (blue color) of the affected regions. Lastly, blood vessels dilate upon rewarming or resolution of the stress, blood returns to the tissues giving them an overly red appearance.[1] Most cases of RP are harmless, self-limited, and do not require any testing, medications, or treatment.[1,2, 4] However, RP can rarely be an early manifestation of other more chronic and serious conditions, most commonly connective tissue diseases (CTD).[1] If the decrease in blood flow and oxygen is severe enough, it can cause irreversible arterial damage, pain, and ulcers; the presence of which should raise suspicion of secondary RP.[2,3,5] Patients presenting with RP in their 3rd to 5th decade are at increased risk for having CTD. In up to 60% of patients with RP beginning in their 4th to 6th decade of life, the etiology is atherosclerotic in nature.[2]
Extra care must be given to the history and physical exam during evaluation of RP in order to differentiate the likelihood of primary versus secondary RP with further testing being recommended for those with signs or symptoms of underlying rheumatologic or atherosclerotic disease.[2]
Key Concepts #
- RP affects 3-21% of the population.
- RP results in discoloration of the fingers and toes in cold weather, or in times of increased emotion and stress.
- The diagnosis is made clinically, often without any further testing.
- Primary RP is a benign self-limited process in most cases (estimated at 80-90% of cases).[6]
- If RP is painful or causes ulcers, a thorough history and physical is recommended to evaluate for signs of associated rheumatologic or cardiovascular disease.
- Rarely, RP can be the early manifestations of more serious and chronic conditions.
- Treatment most often consists of conservative measures including maintenance of whole body warmth, avoidance of cold temperatures and use of gloves and insulated shoes, etc.
- When necessary, treat with topical vasodilating creams containing nitroglycerin or nifedipine, or with oral vasodilating agents such as calcium channel blockers.
Epidemiology #
Raynaud’s phenomenon has been reported to affect 3-21% of the population with most studies indicating a prevalence of 3-5%.[3,5,6] Women are affected more commonly than men. 14-37% of those diagnosed with primary RP will progress to a diagnosis of secondary RP.[6]
Clinical Features #
- The color changes start at the tip of the finger.
- The demarcation is usually clear-cut and involves both the anterior and posterior aspects of the digits.
- There is almost always concurrent numbness at the affected fingertip and paresthesia when warmed.[1,2,5]
- Pain may occur in the affected tissues, and severe ulceration and cutaneous necrosis may occur. If digital ulceration or local tissue damage is present, an underlying cause for the RP should be strongly suspected.[2,5]
- Other sites may also be affected including the nose, ears, tongue, and nipples.[1]
- Because RP is a vasospastic disorder, it may also be seen in association with other vasospastic disorders such as migraine, irritable bowel, microvascular disease and chest pain.[2]
Differential Diagnoses #
- Acrocyanosis
- Erythromelalgia
- Chilblains/pernio
- Paroxysmal digital hematoma
- Peripheral neuropathy
- Occlusive vascular disease
- Vibration disease
- Non-freezing cold injuries, i.e. frostbite.[2]
Diagnostic Workup #
The diagnosis of RP is made clinically based on history, signs and symptoms. In the presence of signs or symptoms concerning for associated rheumatologic disease, CBC and serologic testing should be considered, including ANA, anti-topoisomerase, anti Ro & La, and anti-centromere antibodies. Capillary microscopy can be done and is one of the most sensitive tests in detecting early connective tissue diseases but is not widely available.[2,5]
Treatment #
Primary RP is first treated with conservative measures including avoiding triggers like cold temperature, wearing gloves, utilizing hand and foot warmers and maintaining a warm core. This is adequate for the majority of patients.[3] If these measures fail to adequately control RP, topical vasodilators can be used to prevent the vasospasm from occurring. The most commonly used medications are calcium channel blockers like topical nifedipine, although topical nitroglycerine cream is also used.[3,5] Systemic calcium channel blockers and vasodilators like the phosphodieserase-5 inhibitor sildenafil, along with alpha blockers, botulinum toxin injections, and SSRI’s have been found to be beneficial in the treatment of RP recalcitrant to conservative and topical therapies.[5] Intravenous prostanoid therapy is reserved to treat those whose condition has progressed to digital ulceration or critical ischemia.[5] Additionally, surgical procedures such as fat grafting, surgical debridement, digital sympathectomy, and amputation have been advocated for and used in the treatment of severe RP, though evidence for the effectiveness of these surgical procedures is currently lacking, most likely due to the small number of patients undergoing surgical treatment for this disorder.[5]
Slide Viewer #
https://utahderm.med.utah.edu/image-viewer/References #
- Rigante D, Fastiggi M, Ricci F, Derrico F, Bracci B, Guerriero C. Handy Hints About Raynauds Phenomenon in Children: A Critical Review. Pediatric Dermatology. 2017;34(3):235-239. doi:10.1111/pde.13129.First published:19 May 2017 https://doi-org.ezproxy.lib.utah.edu/10.1111/pde.13129
- Belch J, Carlizza A, Carpentier PH, et al. ESVM guidelines – the diagnosis and management of Raynaud’s phenomenon. Vasa. 2017;46(6):413-423. doi:10.1024/0301-1526/a000661
- Ling SM, Wigley FM. Raynauds Phenomenon in Older Adults. Drugs & Aging. 1999;15(3):183-195. doi:10.2165/00002512-199915030-00002.
- Herrick, A. L. (2019). Raynaud’s phenomenon. Journal of Scleroderma and Related Disorders, 4(2), 89–101. https://doi.org/10.1177/2397198319826467
- Maundrell A., Proudman S.M. (2015) Epidemiology of Raynaud’s Phenomenon. In: Wigley F., Herrick A., Flavahan N. (eds) Raynaud’s Phenomenon. Springer, New York, NY
- Herrick A. L. (2017). Evidence-based management of Raynaud’s phenomenon. Therapeutic advances in musculoskeletal disease, 9(12), 317–329. doi:10.1177/1759720X17740074
- Ling SM, Wigley FM. Raynauds Phenomenon in Older Adults. Drugs & Aging. 1999;15(3):183-195. doi:10.2165/00002512-199915030-00002.
