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  • • 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

  • • Corticosteroids for scleroderma may slow disease

References

Knapid-Kordecka M et ...

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