Background: Urticarial vasculitis is a form of cutaneous leukocytoclastic vasculitis clinically characterized by persistent and often painful urticarial lesions. Numerous systemic diseases have been associated with urticarial vasculitis, including certain hematologic disorders. This distinctive form of cutaneous necrotizing vasculitis can be resistant to standard therapeutic modalities, necessitating more aggressive intervention.
Methods: We report a case of refractory urticarial vasculitis developing in association with B-cell chronic lymphocytic leukemia in a 46-year-old man. We also reviewed the literature to identify other cases of urticarial vasculitis managed with this therapeutic modality.
Results: The disease progressively improved during 6 treatments with plasmapheresis (plasma exchange). In the additional cases identified in the literature, plasmapheresis was generally effective and well tolerated.
Conclusion: On the basis of these findings, we propose that plasmapheresis be considered a treatment option for refractory urticarial vasculitis.