Author: Dr Paul Maurice MB BChir FRCP MD, Dept of Dermatology, Christchurch, New Zealand, 2004.
Raynaud phenomenon is an episodic reduction in the blood supply to the fingers and/or toes occurring mainly in response to cold (vasospastic disease). It can be divided into two main types:
An attack of Raynaud phenomenon is triggered by exposure to cold, such as going out into a cold wind or immersing the hands in cold water. Sudden emotional or psychological upsets can also bring on an attack. Typically, one or more fingers will turn white and numb and, on rewarming, blue due to a sluggish blood flow. This is then sometimes followed by a bright red colour due to a compensatory increased blood flow before the normal skin colour and sensation are restored. Attacks may be painful and can last from minutes to hours. The condition most commonly affects the hands, but sometimes involves the feet and occasionally the tip of the nose or the earlobes.
Primary Raynaud phenomenon is about twice as common as the secondary form. It usually starts under 25 years of age and is five times commoner in women than in men. Individuals often recall cold intolerance dating back to childhood.
Patients with secondary Raynaud phenomenon will often have the symptoms of an associated condition (see below) or be on medication that can cause Raynaud phenomenon as a side effect. If you develop Raynaud phenomenon for the first time when you are older than 25 years and do not recall any cold intolerance in childhood, then it is particularly important that you seek medical advice in order to exclude any underlying causes.
Secondary Raynaud phenomenon is much less common than the primary form, but can be associated with a variety of underlying conditions. The commoner causes are as follows:
You should seek medical advice for the following reasons:
In the first instance your general practitioner will probably refer you to a dermatologist, or possibly a rheumatologist, who will examine you and may arrange some blood tests. You may also have a special examination of the small blood vessels in your nail cuticles, known as nail fold capillary microscopy, which helps to distinguish primary from secondary Raynaud phenomenon. On the basis of these investigations, your dermatologist should be able to advise you on whether you have primary or secondary Raynaud phenomenon and, if secondary, will be able to recommend treatment for the associated disorder.
Raynaud phenomenon may persist for a long time or may improve spontaneously. There are various measures that you can take yourself which can help the condition.
Your dermatologist will be able to give you a lot of advice about your Raynaud phenomenon and the general measures that can be taken to help it.
Any underlying disease, or predisposition to such a disease, will be diagnosed and treated accordingly.
Some drugs can be helpful, especially in primary Raynaud phenomenon. The most effective are those known as calcium channel blockers, such as nifedipine or diltiazem. Verapamil appears ineffective. The calcium channel blockers act by dilating the small blood vessels, thereby increasing the blood flow to the peripheries. The dose may be gradually increased if necessary but it is important that blood pressure is monitored. Sildenafil (Viagra™) has also been reported to be of benefit.
There are promising reports that botulinum toxin injections into the neurovascular bundles at the palmar arch at the base of the digits result in rapid reduction in pain and can heal digital ulcers.
A very small minority of patients with severe and intractable Raynaud phenomenon may require a sympathectomy. This involves cutting the nerves that constrict the peripheral blood vessels. However the procedure is not without complications and has a fairly low success rate, so should only be considered if all else has failed.
Very severe disease that is resulting in ulceration or resorption of the fingertips may be treated by an intravenous infusion of prostacyclin. This requires admission to hospital for a few days.
Other agents reported to be helpful include topical nitroglycerin, topical L-arginine, and losartan. In patients with low blood pressure, oral L-arginine, vitamin E, low-dose aspirin, dypyridamole, niacin and pentoxifylline may be tried.
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