Raynaud's Phenomenon
The Raynaud’s phenomenon (RP) is defined by sharply demarcated color change of the skin of the digits and toes in response to cold or emotional stress. Episodic vasospasm and occlusion of the digital arteries is thought to underlie the disorder.
Risk factors
It is more common among young women (onset often in teens), younger age groups (less than 40), and family members of patients with RP and in regions with colder climate.Prevalence of primary RP (PRP) is 3-16% among women and 1-6% among men.Secondary RP (SRP) usually occurs at older ages (above 40) with a male preponderance. Spontaneous remission of primary RP can occur.
Signs and symptoms
There are two types of Raynaud’s Phenomenon: primary and secondary to another disease/cause.
RP is considered primary if it occurs alone or secondary if it occurs in the presence of a related illness (systemic lupus erythematosus, scleroderma, rheumatoid arthritis, Sjogren’s Syndrome, Polymiositis, vasculitis) or induced by a drug (beta-blockers, ergot alkaloids, cocaine), or by vibration in workers with a vibratory tool or if caused by an occlusive arterial disease (arteriosclerosis or antiphospholipid syndrome), vascular trauma or after frostbite.
RP occurs in attacks of sudden onset of finger and/or toe pain, cold, sensation of pins and needles, numbness and/or clumsiness with a sharply demarcated sequential color change of the skin from white (pallor) to blue (purple) with cold exposure as a result of low blood flow in the small blood vessels of the fingers.
In uncomplicated primary or idiopathic Raynaud’s, this ischemic phase is reversible being followed by a red recolloration with warm exposure.
In secondary RP, severe tissue ischemia may result in pain and necrosis (ulceration) of the skin (usually tips of the fingers and toes). These attacks usually begin in a single finger and then spread to other digits often sparing the thumbs. Both hands and feet (symmetric) as well as other sites like the skin of the nose, ears, face, knees and nipples can be involved. Sometimes a mottling of the skin, known as livedo reticularis can be seen in patients with RP, completely reversible with rewarming.
Treatment
- Patient education: Avoid cold exposure, keep whole body warm, avoid rapidly changing temperatures. Reduce emotional stress. Avoid smoking. Avoid vasoconstricting drugs: decongestants, amphetamines, some cold medicine. Avoid use of vibrating tools. Know techniques to terminate an attack: by placing the hands under warm water or in a warm place (such as the armpits), or rotating arms in a whirling or windmill pattern.
- Medical treatment: Medications that keep the vessels open (vasodilators): calcium channel blockers, nitroglycerine, minoxidil, hydralazine, sildenafil, prostaglandins (iloprost). Patients with a severe ischemic attack might require hospitalization to keep them warm and quiet, with adequate pain control, medical treatment with vasodilators, blood thinners in thrombotic events (ASA, Heparin) and/or chemical (digital or regional lidocaine block) or surgical sympathectomy.