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Recurrent Macular Vasculitis in Hypergammaglobulinemia as an Underrecognized Presenting Manifestation of Activated
Alexa G Ries1, Katherine L Vaidy2, Mariko Suchi3
1Department of Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Abstract:
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.
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