A 34-year old male comes to office complaining of bilateral tingling and pain in his hands, which becomes more intense during exercise. He has also noticed that his fingers turn white and become numb, when exposed to cold. He is an active heavy smoker, drinks occasionally and denies illicit drug use. The patient's medical history is unremarkable apart from recurrent, small and self-limited lower limb ulcerations since his 30s, involving mainly the toes. He denies gross hematuria, bloody stools, cough and shortness of breath. During the clinical examination the hands are found to be cool, mildly edematous and the tips of the fingers have a black discoloration. The Allen test is performed, and it is abnormal for both hands. Lung auscultation shows bilateral fine crackles, while the S1 and S2 heart sounds are normal without any murmurs. Abdominal examination is unremarkable. The blood pressure of the patient is 131/79 mmHg, his heart rate is 68 bpm and he his respiration rate is 14 breaths per minute.
The correct answer is: C. Thromboangiitis obliterans
Buerger's disease (i.e. Thromboangiitis obliterans) is a non-atherosclerotic vasculitis. It has a higher prevalence among young males, who are usually younger than 45 years of age at the time of symptom onset. This vasculitis usually affects small or medium-sized arteries and veins in the upper and lower extremities, starting from the small distal vessels. Eventually distal ischemia develops and as such most patients present complaining of rest pain at their extremities or intermittent claudication.1,2 Moreover, in almost 40% of the patients. secondary Raynaud's phenomenon is observed, while in some cases digital or toe ulcerations are apparent. Although, its cause remains unknown, it has been associated with heavy tobacco use.1 When there is a high suspicion of Buerger's disease, the Allen test should be performed to assess the circulation in the hands. An abnormal Allen test in a young smoker, especially when there are concomitant leg ulcers, is highly indicative of thromboangiitis obliterans, since it demonstrates small-vessel involvement in both the arms and the legs.
This patient does not have characteristic findings of SLE such as fever, fatigue, joint pain, mucosa ulcers, anemia and renal involvement. (Answer A)
Takayasu arteritis mainly affects the aorta and its major branches. Thus, it does not involve the small and medium-sized arteries in the upper and lower extremities, as in this case. Manifestations like ulcerations in the extremities, Raynaud phenomenon, resting pain and paleness in a young, male, heavy smoker make Buerger's disease a more likely diagnosis. (Answer B)
Leukocytoclastic vasculitis typically refers to small-vessel vasculitis of the skin. It classically presents with purpuric papules being a few millimeters in diameter, located predominately on the lower legs, thighs, buttocks, and lower abdomen. Vesicles, pustules, and urticarial papules are sometimes seen. Other symptoms such as fever, abdominal pain, numbness or tingling of the hands or feet, presence of gross hematuria or bloody stools, cough and shortness of breath, may accompany leukocytoclastic vasculitis. This patient does not have similar cutaneous findings and lacks systemic symptoms. (Answer D)
Antiphospholipid syndrome (APS) is an autoimmune disease presented mostly in young women. It causes a hypercoagulable status and as such patients are prone to clot formation. They usually present with DVT, stroke, transient ischemic attack, repeated miscarriages and rash. This patient has none of the above listed symptoms and his age and sex are not typical of APS. Apart from that, the patient's recurrent lower limb ulcerations indicate Buerger's disease as a more likely diagnosis. (Answer E)
Although this patient has increased risk of having atherosclerosis, the chance of that patient having simultaneous atherosclerotic emboli in both upper arms is minimal. (Answer F)
Educational Objective:
Thromboangiitis obliterans is an inflammatory vascular disease predominantly affecting small and medium sized arteries and veins. Symptoms usually ranges from intermittent claudication to pain at rest due to severe ischemia. Recurrent ulcerations and gangrene in the distal extremities are often present.2 Although the cause of Buerger's disease is unknown, there is strong association with heavy tobacco use.
References
- Del Conde I, Peña C, Buerger disease (thromboangiitis obliterans). Tech Vasc Interv Radiol 2014;17:234-40.
- Olin JW, Thromboangiitis obliterans (Buerger's disease). N Engl J Med 2000;343:864-69.
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