Medium-Sized Arterial Vasculitis Associated with Vascular Deposits of Immunoglobin E. Favorable Response to Intravenous Methylprednisolone and Cyclophosphamide

Herein we describe the case of a female patient with medium-sized arterial vasculitis associated with high serum immunoglobin E (lgE) levels in the absence of eosinophilia. During occlusive vascular events, IgE deposits in the arterial muscle layer were demonstrated by immunohistochemistry, suggesting a pathogenic role of this antibody in blood vessel inflammation. Combined treatment with methylprednisolone and cyclophosphamide was successful in reducing serum IgE levels and IgE tissue deposits and in inducing the clinical manifestations of vasculitis into remission. This primary medium-sized IgE-associated vasculitis may constitute a new syndrome not previously reported.