Vasculitis and Vasculopathy

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🌱 來自: Huppert’s Notes

Vasculitis and Vasculopathy🚧 施工中

Vasculitis and Vasculopathy

•   Leukocytoclastic vasculitis (LCV): Histopathologic term that describes nuclear debris from an inflammatory infiltrate; often used interchangeably with small vessel or cutaneous vasculitis

Cutaneous small vessel vasculitis (CSVV)

•   Pathophysiology: Skin-isolated small vessel vasculitis without systemic vasculitis or glomerulonephritis; typically with LCV histology; most often idiopathic (~50%) but can be caused by infection (HBV, HCV, bacteremia), autoimmune disease, or drug-induced (penicillins, cephalosporins, sulfa drugs, diuretics)

•   Clinical features: Presents with palpable purpura (non-blanching purple papules) in dependent areas (lower extremities, buttocks, posterior thigh)

•   Treatment: Eliminate potential triggers, leg elevation, compression stockings, topical steroids

Cholesterol emboli

•   Pathophysiology: Vasculopathy due to arterial embolization of cholesterol crystals

•   Clinical features: Presents with retiform (“net-like”) purpura or livedo reticularis in the distal lower extremities; classically occurs after endovascular procedures or thrombolysis

•   Treatment: Supportive care

Cryoglobulinemia

•   Pathophysiology: Small- and medium-vessel clot formation (vasculopathy) and inflammation (vasculitis) due to precipitation of intravascular proteins (cryoglobulins); associated with HCV, autoimmune diseases, and plasma cell dyscrasias (MGUS, multiple myeloma, Waldenstrom macroglobulinemia)

•   Clinical features: Presents with stellate (“star-like”), reticular, or palpable purpura which may progress to ulcers, ischemia, or necrosis. Typically occurs on the lower legs, feet, digits, and ears due to exposure to colder temperatures

•   Treatment: Treat underlying disease

Calciphylaxis

•   Pathophysiology: Progressive vascular calcification most commonly due to ESRD (uremic calciphylaxis), although also can be seen in hyperparathyroidism, malignancy, and with warfarin use

•   Clinical features: Exquisitely painful purpuric lesions with associated ischemia, necrosis, and bullae

•   Treatment: Pain management, wound care, remove potential triggers, sodium thiosulfate, dialysis if appropriate