Characteristic cutaneous manifestations: |
- Subcutaneous nodules, livedo reticularis, ulcers, retiform purpura, digital ischemia
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Consistent histopathologic findings: |
- Fibrinoid necrotizing vasculitis of medium-sized arteries in the deep dermis/subcutis
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Absence of: |
- Fever ≥38°C for ≥2 weeks, unexplained weight loss ≥4 kg
- Hypertension (new-onset diastolic blood pressure >90 mmHg)
- Progressive renal failure (serum blood urea nitrogen >40 mg/dL or creatinine >1.5 mg/dL)
- Cerebral ischemia, cerebral hemorrhage
- Myocardial infarction, ischemic heart disease, pericarditis, heart failure
- Intestinal hemorrhage, intestinal infarction
- Peripheral neuropathy remote from cutaneous lesions
- Arthralgia (arthritis) or myalgia (myositis) remote from cutaneous lesions
- Abnormal arteriography (microaneurysms, stenosis, or occlusion of visceral arteries)
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