A smoker who sustained finger trauma now presents with Raynaud phenomenon in both hands and generalized itching—what is the most likely diagnosis and how should it be managed?

Buerger's Disease (Thromboangiitis Obliterans)

This clinical presentation—a young male smoker with finger trauma followed by bilateral Raynaud phenomenon and generalized pruritus—is highly suggestive of Buerger's disease (thromboangiitis obliterans), and immediate smoking cessation is the single most critical intervention to prevent disease progression and limb loss. 1, 2

Clinical Diagnosis

The diagnosis of Buerger's disease is primarily clinical and requires meeting specific criteria while excluding other conditions:

Diagnostic Criteria (All Must Be Present)

• Age of onset <45-50 years 1, 3
• Current or heavy tobacco use (this patient is a smoker) 1, 4
• Distal extremity ischemia (bilateral Raynaud phenomenon affecting both hands) 1, 5
• Infrapopliteal/distal arterial occlusions with sparing of proximal vessels 1
• Absence of atherosclerotic risk factors (diabetes, hyperlipidemia, hypertension) 1, 3
• Exclusion of autoimmune disease, hypercoagulable states, and embolic sources 1, 3, 2

Key Clinical Features Supporting This Diagnosis

• Superficial thrombophlebitis (migrating thrombophlebitis is distinctive for Buerger's vs. other vasculitides) 4
• Bilateral upper extremity involvement with Raynaud phenomenon or digital ulceration 1
• Trauma as precipitating event (finger trauma can unmask underlying vascular insufficiency) 5

Important caveat: The generalized itching is unusual for classic Buerger's disease and warrants investigation for alternative or concurrent diagnoses, particularly polycythemia vera, which can present with both peripheral vascular symptoms and aquagenic pruritus. 6, 7

Mandatory Diagnostic Work-Up

Laboratory Screening to Exclude Mimics

First-tier tests (all required): 7

• Complete blood count with differential (to exclude polycythemia vera, which can cause both Raynaud's and pruritus) 6, 7
• Comprehensive metabolic panel (exclude diabetes and renal disease) 7
• Fasting glucose or HbA1c (diabetes exclusion is mandatory) 1, 3
• Liver function tests (exclude hepatic causes of pruritus) 7
• Thyroid-stimulating hormone 7
• Erythrocyte sedimentation rate/C-reactive protein (exclude systemic vasculitis) 7
• Antinuclear antibody, rheumatoid factor (exclude connective tissue disease) 1, 2
• Hypercoagulability panel (anticardiolipin antibodies, lupus anticoagulant, protein C/S, Factor V Leiden) 1, 3

If polycythemia suspected (elevated hemoglobin/hematocrit):

• JAK2 V617F mutation analysis (present in up to 97% of polycythemia vera cases) 7
• Peripheral blood smear 7

Vascular Imaging

Angiography (CT angiography or conventional) is essential to demonstrate: 1, 4

• Segmental occlusions of small-to-medium arteries in distal extremities 1, 4
• "Corkscrew" collaterals (characteristic but not pathognomonic) 1
• Sparing of proximal vessels (no atherosclerotic changes in aorta, iliac, or proximal limb arteries) 1
• Smooth, tapered occlusions without atherosclerotic irregularity 4

Upper extremity arterial duplex ultrasound is appropriate for initial evaluation of suspected positional arterial obstruction and digital ischemia. 6

Immediate Management

Smoking Cessation (Non-Negotiable)

Complete tobacco abstinence is the only intervention proven to halt disease progression and prevent amputation. 1, 4, 3, 2

• Tobacco cessation generally results in disease quiescence 1
• Continued smoking leads to relentless progression with 40-50% amputation rates 2
• All forms of tobacco (cigarettes, cigars, chewing tobacco, e-cigarettes) must be eliminated 2
• Refer to smoking cessation program with pharmacotherapy (varenicline or combination nicotine replacement) 2

Pharmacologic Therapy

Intravenous prostanoid infusions (iloprost) are the primary medical therapy: 5, 2

• Iloprost 0.5-2 ng/kg/min IV infusion over 6 hours daily for 14-28 days 5, 2
• Improves pain, promotes ulcer healing, and reduces amputation risk 5, 2

Adjunctive oral medications: 5, 2

• Pentoxifylline 400 mg three times daily (improves microcirculatory flow) 5
• Calcium channel blocker (e.g., nifedipine 30-60 mg daily for Raynaud's) 4, 5
• Antiplatelet therapy: aspirin 81-100 mg daily or clopidogrel 75 mg daily 5, 2
• Low molecular weight heparin during acute flares, transition to oral anticoagulation if thrombophlebitis present 5

Pain Management

Effective analgesia is crucial for ischemic and neuropathic pain: 2

• Start with gabapentin 300-900 mg three times daily for neuropathic component 2
• Opioids may be necessary for severe ischemic pain 2
• Consider spinal cord stimulation for refractory pain 2

Management of Pruritus

If pruritus persists after addressing vascular disease: 6, 7

• High-lipid content emollients applied liberally 7, 8
• Non-sedating antihistamines (fexofenadine 180 mg daily or loratadine 10 mg daily) 7, 8
• Avoid sedating antihistamines (increased fall risk and cognitive impairment) 7, 8
• If polycythemia vera confirmed: aspirin 300 mg daily specifically treats aquagenic pruritus 6

When Revascularization Is Not an Option

Surgical revascularization plays a limited role due to distal, diffuse disease: 2

• Bypass procedures rarely feasible (distal target vessels typically occluded) 4, 2
• Consider only if adequate distal target vessel identified on angiography 4
• Emerging therapies: immunoadsorption, autologous stem cell therapy, bosentan (investigational) 2

Critical Pitfalls to Avoid

1. Do not delay smoking cessation counseling—this is more important than any medication 1, 3, 2
2. Do not miss polycythemia vera—check CBC and JAK2 mutation if hemoglobin elevated, as this explains both vascular symptoms and pruritus 6, 7
3. Do not use sedating antihistamines for pruritus management 7, 8
4. Do not attribute all symptoms to Buerger's—generalized pruritus warrants systemic work-up for hematologic malignancy, hepatic disease, or drug reaction 6, 7
5. Do not perform extensive cancer screening unless history and physical examination suggest specific malignancy 7

Prognosis and Follow-Up

• With complete smoking cessation: disease typically becomes quiescent, amputation risk <5% 1, 2
• With continued smoking: progressive ischemia, 40-50% require amputation within 5-10 years 2
• Mortality is not increased (coronary, cerebral, and visceral circulations typically spared) 1
• Integrated multidisciplinary care (vascular surgery, pain management, wound care, social work) improves outcomes 2