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7 Cards in this Set

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Acrocyanosis
is persistent, painless, symmetric cyanosis of the hands, feet, or face caused by vasospasm of the small vessels of the skin in response to cold.

Acrocyanosis usually occurs in women and is not associated with occlusive arterial disease. The digits and hands or feet are persistently cold and bluish, sweat profusely, and may swell. In acrocyanosis, unlike Raynaud's phenomenon, cyanosis persists and is not easily reversed, trophic changes and ulcers do not occur, and pain is absent. Pulses are normal.

Treatment, other than reassurance and avoidance of cold, is usually unnecessary. Vasodilators may be tried but are usually ineffective.
Erythromelalgia
is distressing paroxysmal vasodilation of small arteries in the feet and hands and, less commonly, in the face, ears, or knees; it causes burning pain, increased skin temperature, and redness.

This rare disorder may be primary (cause unknown) or secondary to myeloproliferative disorders (eg, polycythemia vera, thrombocythemia), hypertension, venous insufficiency, diabetes mellitus, SLE, RA, lichen sclerosus, gout, spinal cord disorders, or multiple sclerosis.

Burning pain, heat, and redness in the feet or hands last a few minutes to several hours. In most patients, symptoms are triggered by warmth (temperatures of 29 to 32° C) and are typically relieved by immersion in ice water. Trophic changes do not occur. Symptoms may remain mild for years or become severe enough to cause total disability. Generalized vasomotor dysfunction is common, and Raynaud's phenomenon may occur.

Diagnosis is clinical. Testing is done to detect causes. Because erythromelalgia may precede a myeloproliferative disorder by several years, repeated blood counts may be indicated. Differential diagnosis includes posttraumatic reflex dystrophies, shoulder-hand syndrome, peripheral neuropathy, causalgia, Fabry's disease, and bacterial cellulitis.

Treatment is warmth avoidance, rest, elevation of the extremity, and application of cold. For primary erythromelalgia, gabapentinSome Trade Names
NEURONTIN
Drug Information
or a prostaglandin analog (eg, misoprostolSome Trade Names
CYTOTEC
Drug Information
) may be of benefit. For secondary erythromelalgia, the underlying disorder is treated; aspirin may be helpful when a myeloproliferative disorder is involved.
Fibromuscular dysplasia
includes a heterogenous group of nonatherosclerotic, noninflammatory arterial changes, causing some degree of vascular stenosis, occlusion, or aneurysm.

Fibromuscular dysplasia usually occurs in women aged 40 to 60. The cause is unknown. However, there may be a genetic component, and smoking may be a risk factor. Fibromuscular dysplasia is more common among people with certain connective tissue disorders (eg, Ehlers-Danlos syndrome type 4, cystic medial necrosis, hereditary nephritis, neurofibromatosis).

Fibromuscular dysplasia is usually asymptomatic regardless of location. Symptoms, when they occur, vary by location: claudication in the thighs and calves, femoral bruits, and decreased femoral pulses when leg arteries are affected; secondary hypertension when renal arteries are affected; transient ischemic attack or stroke symptoms when carotid arteries are affected; aneurysmal symptoms when intracranial arteries are affected, and, rarely, mesenteric ischemic symptoms when intra-abdominal arteries are affected.

Definitive diagnosis is made by angiography showing a beaded appearance (in medial or perimedial dysplasia) or a concentric band or long smooth narrowing (in other forms).

Treatment varies by location. It may involve percutaneous transluminal angioplasty, bypass surgery, or aneurysm repair. Smoking cessation is important. Control of other risk factors for atherosclerosis (hypertension, dyslipidemia, diabetes) helps prevent accelerated development of arterial stenoses.
Peripheral arterial aneurysms
are abnormal dilations of the peripheral arteries caused by weakening of the arterial wall

Peripheral arterial aneurysms are usually asymptomatic at the time of detection. Extremities may be painful, cold, pale, paresthetic, or pulseless because of thromboemboli or, rarely, aneurysm rupture. Infectious aneurysms may cause local pain, fever, malaise, and weight loss.

Diagnosis is by ultrasonography, magnetic resonance angiography, or CT. Popliteal aneurysms may be suspected when physical examination detects an enlarged, pulsatile artery; the diagnosis is confirmed by imaging tests.
Peripheral arterial disease (PAD)
also called peripheral vascular disease, is atherosclerosis of the lower extremities causing ischemia. Mild PAD may be asymptomatic or cause intermittent claudication; severe PAD may cause rest pain with skin atrophy, hair loss, cyanosis, ischemic ulcers, and gangrene. Diagnosis is by history, physical examination, and measurement of the ankle-brachial index. Treatment of mild PAD includes risk factor modification, exercise, antiplatelet drugs, and cilostazol or possibly pentoxifylline as needed for symptoms. Severe PAD usually requires angioplasty or surgical bypass and may require amputation. Prognosis is generally good with treatment, although mortality rate is relatively high because coronary artery or cerebrovascular disease often coexists.
Raynaud's phenomenon (Raynaud's disease)
is vasospasm of parts of the hand in response to cold or emotional stress, causing reversible discomfort and color changes (pallor, cyanosis, erythema, or a combination) in one or more digits. Occasionally, other acral parts (eg, nose, tongue) are affected. The disorder may be primary or secondary. Diagnosis is clinical; testing focuses on distinguishing primary from secondary disease. Treatment of uncomplicated cases includes avoidance of cold, biofeedback, smoking cessation, and, as needed, vasodilating Ca channel blockers (eg, nifedipine) or prazosin.
Thromboangiitis obliterans
is inflammatory thrombosis of small and medium-sized arteries and some superficial veins, causing arterial ischemia in distal extremities and superficial thrombophlebitis. Tobacco use is the primary risk factor. Symptoms and signs include claudication, nonhealing foot ulcers, rest pain, and gangrene. Diagnosis is by clinical findings, noninvasive vascular testing, angiography, and exclusion of other causes. Treatment is cessation of tobacco use; prognosis is excellent when tobacco use is stopped, but when it is not, the disorder inevitably progresses, often requiring amputation.