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Raynaud's Disease

Raynauds Disease

Raynaud's disease is a vasospastic disorder causing recurrent vasospasms of the fingers and toes, which is not associated with another illness. Stress or cold exposure may act as triggers.


Presentation

Raynaud’s disease causes episodes of vasospasm during conditions of emotional stress or extreme cold climatic conditions. Usually the toes and fingers are the primary parts that are affected; however in certain instances, the lips, nipples or nose may also get affected. The attacks are short lived but can occur frequently. The following are the various signs and symptoms of Raynaud’s disease.

  • The affected area turns white, followed by blue and then red color.
  • The area is painful to touch, is numb and also tender.
  • As the blood flow gradually returns to normal, the area may throb or tingle and can even produce a burning sensation.
  • The order of color changes as described here may not be the same for all individuals. 
Raynaud Phenomenon
  • Abstract The 'filterability' and electrophoretic mobility of erythrocytes from 42 patients with systemic sclerosis and Raynaud's phenomenon were studied and compared with the findings from 24 patients with Raynaud's disease and 26 normal controls.[ncbi.nlm.nih.gov]
  • Clinical manifestations and diagnosis of the Raynaud phenomenon. . Accessed Aug. 20, 2014. Questions and answers about Raynaud's phenomenon. National Institute of Arthritis and Musculoskeletal and Skin Diseases. . Accessed Aug. 20, 2014. Wigley FM.[web.archive.org]
Gangrene
  • Raynaud’s disease can be a primary disorder or it can develop as the result of conditions such as: Systemic lupus erythematosus Other Connective tissue disorders Raynaud’s disease can lead to: Necrosis Gangrene of the toes Gangrene of the fingertips Extreme[disability-benefits-help.org]
  • These sores may progress to gangrene. In rare cases, gangrene may lead to finger amputation. Living with Raynaud’s phenomenon For most people living with Raynaud’s, it is more of an inconvenience than a serious problem.[hopkinsmedicine.org]
  • The condition is primarily considered benign and in very rare circumstance will the spasm lead to tissue loss (ulceration or gangrene).[evergreenhealth.com]
  • […] by recurrent spasm of the capillaries and especially those of the fingers and toes upon exposure to cold, that is characterized by pallor, cyanosis, and redness in succession usually accompanied by pain, and that in severe cases progresses to local gangrene[merriam-webster.com]
Toe Pain
  • When Raynaud's syndrome is associated with scleroderma (Systemic Sclerosis) problems may involve skin ulcers (sores) or gangrene (tissue death) in the fingers or toes. Painful ulcers and gangrene are fairly common and can be difficult to treat.[medic8.com]
  • Some of the most frequently experienced symptoms associated with crooked toes include: Toe pain or irritation Skin thickening, callus or corn formation Inflammation and redness Toe contracture and resulting joint stiffness or fusion Treatment The first[nwfootankle.com]
Turkish
  • Author information 1 Department of Physical Medicine and Rehabilitation, Gülhane Military Medical Academy, Turkish Armed Forces Rehabilitation Center, Ankara, Turkey. serdarkb@gmail.com Abstract Complex regional pain syndrome (CPRS) and Raynaud's disease[ncbi.nlm.nih.gov]
Coronary Atherosclerosis
  • Impaired endothelium-dependent dilation occurs in other diseases associated with vasospasm, such as coronary atherosclerosis.[ncbi.nlm.nih.gov]
Cyanosis
  • The typical acute attack comprises sequential phases of digital pallor, cyanosis, and rubor induced by cold or emotive stimuli.[jamanetwork.com]
  • Attacks were defined as occurring if two of the possible three color changes (pallor, cyanosis, and rubor) occurred, and serial photographs were scored by three independent raters.[ncbi.nlm.nih.gov]
  • The condition often develops in 3 distinct stages defined as vasoconstriction (initial white coloration), accompanied by cyanosis (development of blue color) and finally rapid blood flow that causes development of red color.[symptoma.com]
  • Ray·naud's disease \ rā-ˈnōz- \ Medical Definition of Raynaud's disease : a vascular disorder that is marked by recurrent spasm of the capillaries and especially those of the fingers and toes upon exposure to cold, that is characterized by pallor, cyanosis[merriam-webster.com]
Vascular Disease
  • The patients developed cold intolerance secondary to atherosclerosis or collagen vascular disease. The results were analyzed using the criteria of ulcer healing and the prevention of amputation.[ncbi.nlm.nih.gov]
  • Characteristically, there is no evidence of atherosclerotic vascular disease in these individuals. Over 90% of Raynaud’s patients have a primary etiology.[evergreenhealth.com]
  • Phase Metabolomic Analysis of Systemic Sclerosis Currently investigators do not have diagnostic and prognostic markers for SSc which almost always starts with a vascular disease (Raynaud's disease) isolated for several years.[centerwatch.com]
  • Incidence and Prevalence of Raynaud's Disease According to the Vascular Disease Foundation, approximately 5 percent of people in the United States have symptoms of Raynaud's disease. Eighty percent of cases occur in women.[healthcommunities.com]
Acrocyanosis
  • Acrocyanosis affects both men and women, and the age at onset is usually between 20 and 45 years. The prognosis of patients with idiopathic acrocyanosis is good, and loss of digital tissue is uncommon.[hyperhidrosis-usa.com]
  • Other vasospastic disorders that should be included in the differential but are usually easily distinguishable by physical examination are acrocyanosis and livedo reticularis. Treatment A.[health.am]
  • To help differentiate benign acrocyanosis from acrocyanosis associated with other serious conditions, the differential diagnosis of unilateral and bilateral acrocyanosis in infants is also discussed.[ped-rheum.biomedcentral.com]
  • See also acrocyanosis. This article was most recently revised and updated by Amy Tikkanen, Corrections Manager.[britannica.com]
  • Acrocyanosis also causes color change of the digits in response to cold but differs from Raynaud syndrome in that it is persistent, not easily reversed, and does not cause trophic changes, ulcers, or pain.[merckmanuals.com]
Corneal Opacity
  • Results of ophthalmic evaluation showed transient corneal opacities. Slit-lamp video photography under cold stress demonstrated conjunctival vascular changes consistent with Raynaud's phenomenon.[ncbi.nlm.nih.gov]
Foot Pain
  • Ball-of-foot pain is one of the most common symptoms associated with hammertoes.[nwfootankle.com]
Fingertip Ulceration
  • Trophic changes of sclerodactyly and, rarely, fingertip ulcerations or gangrene,[jamanetwork.com]
  • People with secondary Raynaud's phenomenon are at risk for fingertip ulcers and tend to have active symptoms all year long.[orthop.washington.edu]
  • Care of the nails must be done carefully to avoid injuring sensitive toes and fingertips. Ulcers on the tips of the digits should be monitored closely by the doctor. These can become infected.[medicinenet.com]
Yellow Nails
  • A case of the yellow nail syndrome with associated bronchiectasis, arterial insufficiency and Raynaud's disease is presented. A brief review of some of the literature on the yellow nail syndrome is made.[ncbi.nlm.nih.gov]
Paresthesia
  • During the trial all patients were subjected to finger photoplethysmography and were given a diary to note daily the number and duration of the crises and presence or absence of pain and paresthesia.[ncbi.nlm.nih.gov]
  • Mild discomfort, paresthesias, numbness, and trace edema often accompany the color changes.[health.am]
  • […] pain, pallor, and prickling; phenomenon applies to secondary symptoms, disease when cause is unknown Intermittent bilateral attacks of ischemia of the fingers or toes and sometimes of the ears or nose, marked by severe pallor, and often accompanied by paresthesia[icd9data.com]
  • Symptoms and Signs Sensations of coldness, burning pain, paresthesias, or intermittent color changes of one or more digits are precipitated by exposure to cold, emotional stress, or vibration. All can be reversed by removing the stimulus.[merckmanuals.com]
Dysesthesia
  • Dysesthesiae and regional diaphoresis may coincide with the ischemic phase, while throbbing pain often accompanies recovery. Trophic changes of sclerodactyly and, rarely, fingertip ulcerations or gangrene,[jamanetwork.com]

Workup

A preliminary history of the disease will be taken followed by a physical examination of the affected areas. In addition, various other laboratory tests and imaging studies would also be carried out to diagnose the condition [7]. These include:

  • Blood tests to determine complete blood count, urea nitrogen, prothrombin time, serum glucose and thyroid stimulating hormones are carried out.
  • Antinuclear antibody test is done to analyze for the presence of antinuclear antibodies. If the test turns out to be positive it indicates a stimulated immune system which is another marker for Raynaud’s disease.
  • Erythrocyte sedimentation rate is necessary to distinguish Raynaud’s disease from secondary Raynaud’s phenomenon. A faster than normal sedimentation rate indicates that there is an underlying disease condition.
Hypocapnia
  • Rewarming of the hands following cold challenge was markedly prolonged in the presence of hypocapnia.[ncbi.nlm.nih.gov]

Treatment

Raynaud’s disease can be treated by adopting simple lifestyle changes. In addition, medications are also employed to reduce the symptoms and prevent future attacks. The following are the various methods for treating Raynaud’s disease:

  • Adopting lifestyle changes: Simple steps such as wearing gloves while handling cold substances or in cold climate can help prevent attacks of vasopasm.
  • Medications: Medications such as calcium channel blockers, alpha blockers and vasodialtors are prescribed for severe cases. In majority of the cases, nifedipine is the usual choice. In many instances, topical nitroglycerin to be applied locally on the affected areas has also been found to be helpful [8].
  • Non-pharmacologic approach: In such type of therapy, patients are advised to avoid foods or conditions that can trigger or generate attacks of Raynaud’s disease. These include avoiding smoking, drugs that can instigate an attack of vasospasm and wearing of appropriate warm clothing in cold climate or while handling frozen stuff [9].
  • Diet: There is small evidence suggesting the use of omega-3 fatty acids contained in fish oils to have positive effect on symptoms. Studies have shown a positive association between consumption of fish oils and improvement in the symptoms of Raynaud’s disease [10].

Prognosis

In majority of the cases, prognosis of Raynaud’s disease is extremely favorable. So far no cases of mortality associated with this disease condition has been noted. However, there have been certain pieces of evidences suggesting that if ischemia of the affected body develops then it can lead to necrosis [6].

Etiology

The exact factors that lead to development of Raynaud’s disease are unknown. However, extreme cold temperatures or stressful conditions can predispose an individual to develop such a condition [3]. Exposure to extreme cold climates or inserting hands in cold water can trigger an attack of Raynaud’s disease. Women are more prone to develop such a condition and those with a family history of this disease are also at an increased risk.

Epidemiology

In the United States, it has been estimated that about 2.2% of women and 1.5% of men per year develop Raynaud’s disease [4]. Globally the rate of prevalence of Raynaud’s disease varies from 4.9% to 20.1% in women, to about 3.8% to 13.5% amongst the men population. The disease usually shows signs of development in individuals aged 20 to 30 years.

Sex distribution
Age distribution

Pathophysiology

In extreme cold temperatures or in conditions of stress, the body parts such as fingers and toes may develop a condition known as vasospasm. This is a condition characterized by narrowing of the arteries that limits blood supply to the affected area.

The condition often develops in 3 distinct stages defined as vasoconstriction (initial white coloration), accompanied by cyanosis (development of blue color) and finally rapid blood flow that causes development of red color. The parts that are most exposed to extreme cold temperatures such as the toes and fingers develop this condition [5].

Prevention

Individuals prone to develop Raynaud’s disease should consider the following to prevent attacks of the disease:

  • Appropriate clothing: Individuals should appropriately dress up during winters. They should also wear warm covering while handling frozen food stuff. While stepping out, it is necessary to wear an extra layer of warmers to negate the effect of cold climate.
  • Relocation: Individuals with severe Raynaud’s disease should consider relocating to warmer climates to prevent attacks of the disease.

Summary

Raynaud’s disease is a rare condition characterized by narrowing of the arteries that carry blood from the heart to different parts of the body. Such a process limits blood circulation to the affected areas [1]. Occurrence of short episodes of vasopasm defined as narrowing of blood vessels mark the development of Raynaud’s disease.

Raynaud’s is basically divided into 2 categories, namely primary and secondary phenomenon. Both these conditions share the same name but have distinct features. The primary Raynaud’s phenomenon is also known as the Raynaud’s disease. This disease was named after the medical student Maurice Raynaud who first defined the characteristics of this condition [2].

Patient Information

Definition

Raynaud’s disease is characterized by attacks of vasospasm that mainly affects the toes and fingers. These are the parts usually exposed to cold climate or extreme conditions. In this condition, the blood supply to the affected area is restricted causing development of vasoconstriction, cyanosis and rapid blood flow. The condition is more common in women than men.

Cause

The exact cause of Raynaud’s disease is unknown. Extreme cold temperatures and conditions of stress are known to induce attacks of vasospasm. The exposed areas of the body, most commonly the toes and fingers are known to get affected.

Symptoms

Symptoms of Raynaud’s disease include color changes of the affected areas. Toes and fingers may first turn white color which may then turn blue. Numbness can also set in.

Diagnosis

Diagnosis consists of blood tests to rule out underlying disease conditions. In addition, antinuclear antibody test is also done to assess the levels of the antibody.

Treatment

Treatment of Raynaud’s disease includes making certain lifestyle changes to manage the symptoms and prevent future attacks. In addition, certain medications are also given such as calcium channel blockers to relieve the symptoms. In many cases, topical application of nitroglycerin is also advised.

References

Article

  1. Block JA, Sequeira W. Raynaud's phenomenon. Lancet 2001; 357:2042.
  2. LeRoy EC, Medsger TA Jr. Raynaud's phenomenon: a proposal for classification. ClinExpRheumatol 1992; 10:485.
  3. Coulombe J, Powell J, Hatami A, McCuaig C, Renet S, Marcoux D. Diseases of abnormal sensitivity to cold in children on psychostimulant drugs. J Cutan Med Surg. Oct 2014;18(5):1-4.
  4. Suter LG, Murabito JM, Felson DT, Fraenkel L. The incidence and natural history of Raynaud's phenomenon in the community. Arthritis Rheum. Apr 2005;52(4):1259-63.
  5. Wigley FM, Flavahan NA. Raynaud's phenomenon. Rheum Dis Clin North Am. Nov 1996;22(4):765-81.
  6. Wigley FM. Clinical practice. Raynaud's Phenomenon. N Engl J Med 2002; 347:1001.
  7. Lavery JP, Lisse JR. Raynaud's Phenomenon. In: Taylor RB, ed. Difficult Diagnosis. Vol 2. Philadelphia, Pa: WB Saunders Co; 1992:386-91.
  8. Ennis H, Anderson ME, Wilkinson J, Herrick AL. Calcium channel blockers for primary Raynaud's phenomenon. Cochrane Database Syst Rev. Jan 30 2014;1:CD002069.
  9. Malenfant D, Catton M, Pope JE. The efficacy of complementary and alternative medicine in the treatment of Raynaud's phenomenon: a literature review and meta-analysis. Rheumatology (Oxford). Jul 2009;48(7):791-5.
  10. DiGiacomo RA, Kremer JM, Shah DM. Fish-oil dietary supplementation in patients with Raynaud's phenomenon: a double-blind, controlled, prospective study. Am J Med. Feb 1989;86(2):158-64.

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Last updated: 2019-07-11 21:44