Upper-Extremity Vascular Disorders
In the spectrum of vascular diseases, upper-extremity arterial problems, including vasospastic disorders, are relatively uncommon. When pain, numbness, coolness, or ulcers involve the fingers or hand, patients generally seek early medical attention. Such symptoms restrict many normal activities and quickly raise the patient’s fear of permanent loss of hand function.
The underlying causes of arm ischemia and vasospastic disorders are diverse. For simplicity, they may be organized into the broad groups of emboli, atherosclerotic or aneurysmal occlusions, trauma, and small-vessel arterial occlusive disease. In the initial evaluation the clinician must determine which group the patient fits into and then diagnostic tests can be selected to define the specific underlying etiology. This chapter emphasizes principles of management after a clinical diagnosis has been established.
COMMON CLINICAL PRESENTATIONS
Although there are multiple etiologies for upper-extremity arterial ischemia, including vasospastic disorders, the clinical presentations are relatively few.
I.
Raynaud’s phenomenon is a description of a clinical presentation or syndrome that suggests vasospasm. The underlying causes are numerous (Table 18.1). Certain clinical features help differentiate Raynaud’s phenomenon from other vasospastic disorders, such as acrocyanosis and livedo reticularis (Table 18.2).
Table 18.1. Etiologies of Raynaud’s phenomenon | |||||||||||||||
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Table 18.2. Differentiation of vasospastic disorders | ||||||||||||||||||||||||||||||||
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A. Raynaud’s phenomenon defines an episodic vasoconstriction of the small arteries and arterioles of the extremities.
The episodes generally are initiated by cold exposure or emotional stimuli. The symptoms of acrocyanosis and livedo reticularis are constant, although they may increase with exposure to cold.
B. The phenomenon usually follows a predictable sequence of color changes in the digits and/ or hand: pallor (i.e., white), followed by cyanosis (i.e., blue), and then rubor (i.e., red).
The pallor occurs when skin perfusion is minimal due to intense vasoconstriction of small arterioles in the hand and digits. As cutaneous flow resumes, it is sluggish, beginning with poorly saturated blood leading to the bluish color of cyanosis. Finally, when cutaneous flow resumes, a hyperemic or reperfusion phase occurs causing the skin to be warm and ruborous. Attacks usually are accompanied by numb discomfort of the fingers although pain generally is not severe unless ulcerations are present. In contrast, acrocyanosis occurs mainly in women and is characterized by continuous bluish discoloration of the hands and occasionally of the lower extremities. Livedo reticularis also is a continuous vasospastic condition consisting of a mottled or reticulated reddish-blue discoloration of the lower extremities and occasionally of the hands.
C.
Raynaud’s phenomenon is localized to the fingers, toes, and occasionally nose and ears. Attacks are limited most commonly to the upper extremities and rarely involve only the toes.
D.
The chance of ulceration or gangrene of the tips of the digits depends on the underlying etiology.
1. Raynaud’s disease is the term applied to Raynaud’s phenomenon that has no clear association with any systemic disease. It rarely results in tissue necrosis and usually occurs in young females (70% of cases). It has been suggested that, before the diagnosis of primary Raynaud’s disease is made, the following criteria should be met:
Bilateral, symmetric Raynaud’s phenomenon is present
There is no evidence of large vessel arterial occlusive disease
There is an absence of gangrene or significant trophic changes
Symptoms should be present for a long period, usually 2 years, without evidence of any other systemic disease associated with Raynaud’s phenomenon.
2. Raynaud’s phenomenon is the term applied when a local or systemic disease appears to be the precipitating factor for this complex of symptoms. In rare cases Raynaud’s phenomenon can also occur in relation to large vessel arterial occlusive lesions in the upper extremity. Gangrene or ulceration of digits is more common in these patients, especially when they have scleroderma, which is the most common collagen vascular disease associated with Raynaud’s phenomenon. Raynaud’s phenomenon is the initial symptom in 30% of cases and eventually affects 80% of patients with scleroderma. The other common rheumatic diseases associated with Raynaud’s phenomenon include mixed collagen vascular disease (80%), systemic lupus erythematosus (30%), dermatomyositis/polymyositis (20%), and rheumatoid arthritis (10%). It must be remembered that Raynaud’s phenomenon may exist for years before some underlying systemic collagen vascular, immunologic disease, or large vessel occlusive disease is diagnosed.
3. Acrocyanosis is not associated with skin ulceration. Livedo reticularis may occur with ulcerations, generally when some systemic disease (e.g., periarteritis nodosa) is present or atherosclerotic microembolism is evident.
II.
Tissue necrosis includes gangrene and poorly healing ulcerations of the digits. It is not uncommon for patients to dismiss small ulcers caused by microemboli as inconsequential bruises or sores of the finger, not recognizing their more serious etiology. If Raynaud’s phenomenon precedes the onset of tissue necrosis, other symptoms and signs of underlying systemic disease should be sought. In the absence of Raynaud’s phenomenon, evidence of large-vessel occlusive disease, emboli, or small-vessel arterial occlusive disease associated with occupational trauma must be sought.
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