Antineutrophil cytoplasmic antibody positivity and cutaneous IgA vasculitis in a patient with antisynthetase syndrome

Vasculitis is a disease process that results from inflammation of blood vessel walls. The clinical presentation is variable and dependent upon the size and location of the vessels involved.1 Cutaneous vasculitis refers to a vasculitis that results in skin manifestations, often due to involvement of small- and medium-sized vessels. The incidence of cutaneous vasculitis ranges from 15.4 to 29.7 cases per 1 million people every year.2 Clinical presentations vary and can include petechiae, purpura, purpuric papules, hemorrhagic vesicles, and bullae.1 Biopsy is the gold standard for making a diagnosis of cutaneous vasculitis.2 The collection of histopathologic findings in cutaneous small-vessel vasculitis are referred to as leukocytoclastic vasculitis (LCV) and include a polymorphonuclear neutrophilic infiltrate, primarily in postcapillary venules, with fibrinoid deposits in and around the vessel wall and extravasation of red blood cells.3 Although LCV is often idiopathic, it may occur secondary to infection, drugs, malignancy, or autoimmune processes, such as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).4 The following case report describes a patient with a history of antisynthetase syndrome (ASS) who subsequently developed IgA vasculitis with copositive ANCA.