A 38-year-old male smoker presented with a 1-year history of left-calf discomfort during ambulation and a 3-month history of progressive bluish discoloration of the toes. He had previously been seen by a vascular surgeon and diagnosed with a ‘near’ occlusion of the tibioperoneal trunk. Physical examination revealed diminished radial artery pulses bilaterally, and the Allen test was positive bilaterally. Tender subcutaneous nodules on the medial aspect of the left calf and a mottled appearance of the skin on the bilateral lower extremities were noted. Computed tomographic (CT) angiography displayed stenosis within the tibioperoneal trunk as well as the infrageniculate arteries bilaterally. Additionally, characteristic “corkscrewing” of distal tibioperoneal and digital arteries was identified. Duplex ultrasonography documented segmental thrombosis of the left great saphenous vein.

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  Initially described by von Winiwarter in 1879, although the eponym was given to Leo Buerger who published extensive pathologic findings from the amputated limbs of afflicted patients in 1908.^1,2

  
  
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  Segmental, nonatherosclerotic inflammatory disease of the small- and medium-sized arteries, veins, and nerves most commonly affecting the hands and feet.^1,2

  
  
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  Typically occurs in young adults (20-45 years old) with a higher incidence in men over women (2:1 ratio).³

  
  
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  There is a high association with current or recent tobacco smokers. There are cases in which thromboangiitis obliterans (TAO) has been associated with chewing tobacco as well as marijuana.^3,4

  
  
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  Prevalence has diminished over the past 5 years on account of decreased smoking as well as adherence to more stringent diagnostic criteria.

  
  
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  Highest incidence occurs in Israeli Jews of Ashkenazi descent and natives of Indian, Korean, and Japanese descent. It is less frequent in subjects of northern European descent.

  
  
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  Death from TAO is unusual, but morbidity is substantial. When affected patients continue to smoke, 43% require one or more amputations in 7.6 years.

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  The definitive etiology of TAO is not known, yet tobacco exposure is required for both disease initiation and progression.

  
  
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  The mechanism of disease remains cryptic but may involve immunologic dysfunction and tobacco hypersensitivity that is associated with enhanced cellular sensitivity to type I and III collagen, impaired endothelium-dependent vasorelaxation, and increased antiendothelial cell antibody titers.

  
  
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  A genetic link may exist as affected subjects have an increased prevalence of human leukocyte antigen (HLA)-A9, HLA-B5, and HLA-54.

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  TAO should be suspected in young (≤45 year old) smokers with a compatible history and symptoms of arterial insufficiency with concurrent superficial venous thrombosis.

  
  
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  Peripheral artery disease (PAD) can mimic TAO and many times presents with similar manifestations and risk factors. Affected populations are typically older than 50 years and may have concurrent coronary artery disease and/or cerebrovascular disease.⁵ However, the combination of arterial insufficiency with superficial venous thrombosis suggests a diagnosis of TAO. In addition, involvement of both upper and lower extremities favors a diagnosis of TAO over atherosclerosis.

Clinical Features

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  Patients with TAO typically present with ischemic signs and symptoms in the distribution of the distal arteries of the upper or lower extremities.³

  
  
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  Manifestations may include claudication (arch of the foot as well as the calf); Raynaud phenomenon (40%) or livedo reticularis; rest pain in hands, feet, and digits; ischemic ulcerations; and/or gangrene (Figures 82-1,Figures 82-2, and 82-3).⁶

  
  
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  Foot or arch claudication is often erroneously diagnosed as a musculoskeletal problem.

  
  
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  Although symptoms of TAO commonly begin in the distal extremities, the disease typically progresses to involve more proximal vessels.

  
  
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  Allen and reverse Allen tests may reveal ulnar or radial artery obstruction, respectively.

  
  
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  Superficial thrombophlebitis complicates almost half of all cases of TAO (Figure 82-4).

  
  
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  Due to associated neurologic involvement, paresthesias of the acral portions of the upper and lower extremities are often described.