Recurrent Macular Vasculitis in Hypergammaglobulinemia as an Underrecognized Presenting Manifestation of Activated <scp>PI3K</scp> Delta Syndrome

Recurrent macular vasculitis in hypergammaglobulinemia, previously termed hypergammaglobulinemic purpura of Waldenström (HGPW), is a rare disorder characterized by recurrent episodes of lower extremity purpura and edema with symptoms of stinging and burning. We report a pediatric case of recurrent macular vasculitis in hypergammaglobulinemia and enteropathy as the presenting clinical manifestation of activated phosphoinositide 3-kinase delta syndrome (APDS). APDS, a rare inborn error of immunity, has been infrequently described in association with vasculitis, and we suspect previously described cases could be more accurately classified as recurrent macular vasculitis in hypergammaglobulinemia.