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Raynaud's Disease:
Definition: Spasm of arterioles, usually in the digits (and occasionally other acral parts such as the nose and tongue), with intermittent pallor or cyanosis of the skin.
Etiology: Raynaud's disease may be idiopathic (Raynaud's disease) or secondary to other conditions: connective tissue disorders (eg, scleroderma, RA, SLE), obstructive arterial diseases (arteriosclerosis obliterans, thromboangiitis obliterans, thoracic outlet syndrome), neurogenic lesions, drug intoxications (ergot and methysergide), dysproteinemias, myxedema, primary pulmonary hypertension, and trauma. Idiopathic Raynaud's disease is most common in young women (60 to 90% of reported cases).
Pathology and Pathophysiology: Attacks of vasospasm of the digital arteries and arterioles may last from minutes to hours but are rarely severe enough to cause gross tissue loss. With long-standing Raynaud's disease, the skin of the digits may become smooth, shiny, and tight, with loss of subcutaneous tissue (sclerodactyly). Small painful ulcers may appear on the tips of the digits. Vessels are histologically normal in early stages, but in advanced cases the arterial intima may thicken and thromboses may form in small arteries. In secondary Raynaud's phenomenon, pathologic changes of the underlying disease are apparent.
Recent developments may broaden our understanding of the pathophysiology of Raynaud's phenomenon and may lead to new approaches to management. Research into prostaglandin metabolism, microcirculation, and the role of the endothelial cell is yielding promising results. Clinical association between Raynaud's phenomenon and migraine headaches, variant angina, and pulmonary hypertension suggests that there is a common mechanism for vasospasm in > 1 arterial bed. The threshold for the vasospastic response in Raynaud's disease is lowered by anything that activates sympathetic outflow or releases catecholamines (eg, emotion) in addition to local cold.
Symptoms, Signs, and Diagnosis: Intermittent attacks of blanching or cyanosis of the digits are precipitated by exposure to cold or by emotional upsets. Color changes may be triphasic (pallor, cyanosis, redness --reactive hyperemia) or biphasic (cyanosis, then reactive hyperemia). Rewarming the hands restores normal color and sensation. Color changes do not occur above the metacarpophalangeal joints and rarely involve the thumb. Pain is uncommon, but paresthesias are frequent during the attack.
Raynaud's disease is differentiated from secondary Raynaud's phenomenon by bilateral involvement, a history of symptoms for >= 2 yr without progression, and no evidence of an underlying cause. In Raynaud's disease, trophic skin changes and gangrene are either absent or present only in minimal areas. In secondary Raynaud's phenomenon, the symptoms and signs of the underlying disease usually become manifest within 2 yr, occasionally longer. In Raynaud's phenomenon associated with scleroderma, there may also be tightness or thickening of the skin and telangiectases of the hands, arms, or face; difficulty swallowing; painful trophic ulcers on the fingertips; and symptoms referable to other systems.
The wrist pulses are usually present, but the Allen test frequently shows occlusion of the radial or ulnar arterial branches distal to the wrist. This is performed by the examiner, who faces the patient and places his thumbs over the radial and ulnar pulsations of one hand. After the patient clenches his fist to expel the blood from the hand, the examiner compresses the arteries. When the patient opens the fist, the hand is pale. The examiner then releases pressure from the radial artery but maintains it on the ulnar artery. If the radial artery distal to the wrist is patent, the hand will rapidly turn pink; if the artery is occluded, the hand will remain pale. The maneuver is then repeated by maintaining pressure on the radial artery while releasing the ulnar artery. Noninvasive testing of the affected digits with plethysmography before and after exposure to cold has particular value in differentiating between occlusive and vasospastic disease in patients with Raynaud's phenomenon.
Treatment: Mild cases of Raynaud's disease may be controlled by protecting the body and extremities from cold and by using mild sedatives (eg, phenobarbital 15 to 30 mg orally tid or qid). The patient must stop smoking since nicotine is a vasoconstrictor. In a few patients, relaxation techniques, such as biofeedback, may reduce vasospastic episodes. Drugs formerly used for treatment have been varied and inconsistently effective. Reserpine 0.1 mg to 0.25 mg orally bid to qid has been commonly used and may decrease the number and severity of attacks, but side effects (eg, depression) may prevent its use. Phenoxybenzamine 10 mg orally qid and methyldopa 1 to 2 gm/day orally have been tried with occasional success. The drugs of choice are prazosin 1 to 2 mg orally at bedtime, and repeated in the morning if necessary, and the Ca antagonist nifedipine 10 to 30 mg orally tid. Reserpine 1.0 mg in 5.0 mL of 0.9% sodium chloride solution injected into the brachial artery q 3 mo may have a beneficial effect on the healing of ulcers. Encouraging reports concerning the effectiveness of pentoxifylline 400 mg bid or tid with meals have appeared in the literature. Therapy of the secondary forms depends on recognition and treatment of the underlying disorder. Research with encouraging results is in progress for the use of prostaglandins (thromboxane) in the treatment of Raynaud's phenomenon. Phenoxybenzamine 10 mg orally qid may be useful. beta Blockers, clonidine, and ergot preparations cause vasoconstriction and may induce or worsen Raynaud's phenomenon and are, therefore, contraindicated.
Regional sympathectomy is reserved for patients with progressive disability; it often abolishes the symptoms, but the relief may last only 1 to 2 yr. Results from sympathectomy are generally better in patients with Raynaud's disease than in those with secondary Raynaud's phenomenon.
REFLEX SYMPATHETIC DYSTROPHY: (Sudeck's Atrophy; Minor Causalgia and Posttraumatic Neuralgia)
This prototype of sympathetically mediated pain occurs following injury to bone and soft tissue. The diagnosis depends on pain associated with autonomic changes (eg, sweating or vasomotor abnormalities) and/or dystrophic changes (eg, skin or bone atrophy, hair loss, joint contractures). Radionuclide bone scan (increased uptake), x-rays of the extremity (bone loss), and thermography (decreased skin temperature) may be useful confirmatory tests, but none need be positive for the pain syndrome to exist.
Causalgia: may be viewed as a subtype of reflex sympathetic dystrophy. In this syndrome, usually partial injury to a nerve trunk (typically the median nerve above the elbow or sciatic nerve above the knee) produces severe, burning pain in the extremity. The pain usually occurs immediately or soon after the injury and in time becomes associated with the autonomic and trophic changes described above.
Treatment: Combined with physical therapy, anesthetic or pharmacologic blockade of sympathetic nerve function is the most important modality. Sympathetic nerve block should be used. Transient relief after repeated temporary blocks suggests the need for surgical or chemical sympathectomy. Regional sympathetic blockade with IV guanethidine or reserpine is a specialized anesthetic technique that may be useful in selected patients. The sympatholytic drugs prazosin (1 to 8 mg/day orally in divided doses) and phenoxybenzamine (40 to 120 mg/day orally in divided doses) have been reported in uncontrolled series to be of benefit. Other suggested drugs include nifedipine (10 to 30 mg orally tid), corticosteroids (eg, prednisone 60 to 80 mg/day orally tapered over 2 to 4 wk), tricyclic antidepressants (see above), and anticonvulsants. Anecdotal reports in postherpetic neuralgia and other neuropathic pain states also suggest benefit from topical application of capsaicin cream 0.025% as well as selected neuroleptics (eg, fluphenazine 1 to 2 mg orally tid). Recently, the analgesic potential of local oral anesthetics (mexiletine 150 to 300 mg tid is preferred) has been explored and a trial in refractory neuropathic pain states is warranted. Chronic treatment with opioid analgesics is controversial, but may occasionally be useful in reliable patients; such therapy should be considered only after all other approaches have failed and if close physician follow- up is possible.
