Vasoconstrictive Disorders

• • Characterized by abnormal lability of sympathetic nervous system

  • Affects arterial and venous side of capillary bed to reduce cutaneous blood flow

  • Sluggish flow of deoxygenated blood causes cutaneous cyanosis, coldness, numbness, pain

Raynaud Syndrome

• • Precipitated by exposure to cold or stress

  • May follow virulent or benign course

Acrocyanosis

• • Persistent cyanosis of hands and feet

  • Occurs in young females

Scleroderma

• • Connective tissue disease: Fibrosis due to increased collagen and elastin, arterial intimal thickening

  • Affects lung, kidney, GI, muscle, CNS

Reflex Sympathetic Dystrophy

• • Post-traumatic pain syndrome

  • Poorly understood

  • Initial injury: Fracture, laceration, crush

  • Unendurable burning pain of entire extremity, extreme sensitivity

  • Increased sympathetic activity is cause

  • Vasoconstriction prominent feature

Epidemiology

Raynaud Syndrome

• • Associated with immunologic and connective tissue disorders (scleroderma, lupus, polymyositis, diabetic arterial disease, trauma, etc)

  • Association with Helicobacter infection reported

Scleroderma

• • More common in women, 25- to 50-years old

Reflex Sympathetic Dystrophy

• • Equally common in men and women; upper = lower extremity

Symptoms and Signs

Raynaud Syndrome

• • Sequence of pallor, cyanosis, rubor (white-blue-red color changes)

Acrocyanosis

• • Cyanosis, numbness, and pain

  • Changes disappear when warm

  • Examine in cold room: Diffuse cyanosis, coldness, and hyperhidrosis symmetrically

  • Allen test may be delayed in cold

  • Pulses normal when warm, absent when cold

Scleroderma

• • First skin and vasculature of hands

  • Skin thick and taut, limited finger flexion

  • Forearm muscles: Woody induration

  • Progressive finger coldness/numbness

  • Painful ulceration of terminal phalanges

  • Allen test: Uneven color return to fingers

Reflex Sympathetic Dystrophy

• • Exquisite pain to minimal stimuli

  • Progressive atrophy

Reflex Sympathetic Dystrophy

• • Sympathetic block often diagnostic

Raynaud Syndrome

• • Helpful to differentiate occlusive lesion from transient vasconstrictive variety

Raynaud Syndrome

• • Avoid cold, tobacco, oral contraceptives, β-blockers

  • Calcium channel blockers, prostaglandins, ketanserin, dilastazol may be helpful

  • Cervical sympathectomy if cause is transient

Scleroderma

• • Palliative, sympathectomy rarely helps

  • Corticosteroids slows progression

  • Amputation if gangrene develops

Reflex Sympathetic Dystrophy

• • Sympathetic block

  • Surgical sympathectomy

  • Spinal cord stimulation, intrathecal baclofen-temporary pain relief

Surgery

Indications

• • Amputation if gangrene develops

Medications