Cutaneous Small Vessel Vasculitis (CSVV)

Figure 3.41 A: CSVV (Courtesy of Dr. Paul Getz) B: CSVV (Courtesy of Dr. Paul Getz) C: Urticarial vasculitis (Courtesy of Dr. Iris K. Aronson)

• General term encompassing diseases with histopathologic features of leukocytoclastic vasculitis (perivascular neutrophilic infiltration and fibrinoid degeneration of vascular walls) involving only small cutaneous blood vessels irrespective of etiology; typically occurs 7 – 10 days after exposure to inciting agent
• CSVV often related to one of the following:
  • Infection (bacterial, viral) – 15–20%
  • Inflammatory disorder (autoimmune connective tissue disease, inflammatory bowel disease, seronegative spondyloarthropathy) – 15–20%
  • Drug-exposure – 10–15%
    • Common: NSAIDs, COX-2 inhibitors, leukotriene inhibitors, penicillins, quinolones, anti-TNF agents, G-CSF, hydralazine, anti-thyroid agents
    • Occasional: ACEI, allopurinol, furosemide, coumarin, quinine, macrolide antibiotics, thiazides, sulfonlyureas, trimethoprim-sulfamethoxazole, vancomycin, IFN, β-blockers
  • Neoplasms – 5%
  • Idiopathic – 50%
• Presents with palpable purpuric to erythematous papules, vesicles, and macules over lower extremities and other dependent areas, ± fever, arthralgias, myalgias and weight loss; extracutaneous involvement typically mild; prognosis depends on severity of systemic involvement
• Histology: leukocytoclastic vasculitis
• Treatment: rule out systemic vasculitis, remove any trigger, supportive therapy (90% spontaneous resolution)