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Therapeutics in Dermatology
A reference textbook in dermatology

Raynaud’s phenomenon

7 May 2019, by MENDES R.

1 - ACKNOWLEDGEMENTS

Chapter written with the help of the EADV, the Fondation René Touraine and the Therapeutics in Dermatology

2 - OVERVIEW

It is the name used to describe digital vasospasm (vessel wall contraction) producing blanching most commonly over the fingers or toes. Smaller arteries that supply blood to the skin narrow, limiting blood circulation to affected areas (vasospasm). It is often triggered by cold exposure and/or emotional stress and is highly prevalent in the general population.

There are two types of Raynaud’s Phenomenon (RP): primary, which accounts for most of all cases of RP, and secondary. Primary RP is characterized by the occurrence of the vasospasm alone, with no association with another illness. Secondary RP is associated with another illness, most commonly an autoimmune disease.

3 - SIGNS AND SYMPTOMS

  • Cold fingers or toes.
  • Colour skin changes: first the skin turns white (blanching) which is usually followed by cyanosis (a blueish colour that appears due to static venous blood flow) and then by redness/rubor (after return of the blood flow), producing a classic triphasic colour change. However, blanching may be the only feature of RP.
  • The colour changes start at the tip of one or more fingers or toes and may spread to involve the entire digit(s). The demarcation of the colour changes is usually clear-cut.
  •  It is almost always associated with numbness of the affected body parts.
  • Besides fingers and toes, other body extremities (like tongue, ear lobes, tip of the nose and the nipples) may be affected.
  • In secondary RP trophic changes may be seen.

4 - WHO GETS RP AND WHAT CAUSES IT?

The cause of Raynaud’s attacks is not completely understood, but blood vessels in the hands and feet appear to overreact to cold temperatures or stress. The affected population depends on the type of RP.

5 - PRIMARY RAYNAUD’S PHENOMENON

  • It is a benign condition affecting 3 to 21% of the population and not associated with an underlying disorder.
  • Typically occurs in young women (between 15 and 30 years) with a familial predisposition.
  • It can be brought on by different stimuli including cold, emotions and carrying objects.
  • It is associated with a high incidence of migraine.
  • It presents as symmetrical vasospasm, producing blanching, usually affecting both hands (thumbs are often spared).
  • Sometimes it may be misdiagnosed as chilblains (perniosis).

6 - SECONDARY RAYNAUD’S PHENOMENON

  • It is associated with underlying systemic disease in more than half of the patients.
  • It may be associated with a connective tissue disorder (particularly systemic sclerosis), an endocrine disorder (like hypothyroidism) or a miscellaneous condition. It may also be a consequence of an occupational exposure/activity or even a result of drugs (like anti-migraine drugs, non-selective β blockers, estrogen replacement therapy without progesterone, ephedrine, cocaine or amphetamine abuse, bromocriptine, cyclosporin and some cytotoxic drugs).
  • Low average daily temperature, stress, anxiety, older age and female gender are associated with an increased rate and severity of RP episodes as well as increased pain.

7 - HOW IS RAYNAUD’S PHENOMENON DIAGNOSED?

RP is diagnosed based on the patient’s history of typical symptoms (well demarcated blanching with or without cyanosis/rubor). Your doctor may ask you about the onset of RP, frequency of attacks and associated symptoms. Taking photographs of the fingers during an attack can often help in the diagnosis. A through examination should also be taken to ensure correct diagnosis of any underlying condition. Other tests are not routinely recommended and are not necessary unless there is any suspicion of a secondary RP. If that is the case further targeted investigations are required and the patient referred to a specialist.

8 - HOW IS IT TREATED?

RP may lead to considerable pain and disability, but there are ways to relief symptoms. Patient education is a key component. If you have questions, ask your doctor or other health care professional. Self-help groups can also be useful. Not all patients require drug treatment. The goal of treatment is to provide symptom relieve and improve quality of life.

General measures, including lifestyle changes and adaptations are key in the management of all patients with RP. If general measures are not enough and symptoms still interfere with patients’ social or working lives, then drug therapy should be considered. There are several drugs available. Usually, longer-acting preparations of vasodilatory drug therapies such as calcium channel blockers are the first line drug treatment. Your doctor will consider all options and treatment decisions are individualized on a case-to-case basis. Some patients may need combination therapy, and in more severe cases, other kinds of treatment may be necessary.

9 - TIPS FOR MANAGING: GENERAL MEASURES FOR PATIENTS WITH RP

  • Avoid cold environments.
  • Protect yourself from cold, for instance, by wearing multiple layers of clothing.
  • Wear gloves when handling frozen food.
  • Move the body.
  • Avoid sudden temperature changes.
  • Avoid emotional stressors whenever possible.
  • Stop smoking! Cigarette smoking promotes vasoconstriction.
  • Avoid vibration exposure.
  • Check with your doctor if you are taking any vasoconstrictor drug. If so, your doctor may consider stopping it, if possible.
  • Help end an attack by warming the affected hands or feet, methods include placing the hands or feet under warm water or in a warm place (such as placing your hands in the armpits).
  • There are valuable assistive devices you can use when symptoms develop to help you to perform tasks with less difficulty. For instance, in cold weather, a key holder may be useful to help you to unlock a door if your fingers get numb.

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