Recurrent Macular Vasculitis in Hypergammaglobulinemia as an Underrecognized Presenting Manifestation of Activated PI3K Delta Syndrome. Pediatr Dermatol 2026;43(2):452-457
Date
11/05/2025Pubmed ID
41190409DOI
10.1111/pde.70097Scopus ID
2-s2.0-105020689821 (requires institutional sign-in at Scopus site)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.
Author List
Ries AG, Vaidy KL, Suchi M, Bolling K, Remiker AS, Austenfeld E, Chiu YEAuthors
Emma Austenfeld MPH, MD Assistant Professor in the Pediatrics department at Medical College of WisconsinYvonne E. Chiu MD Vice Chair, Professor in the Dermatology department at Medical College of Wisconsin
Allison S. Remiker MD Associate Professor in the Pediatrics department at Medical College of Wisconsin
Mariko Suchi PhD, MD Professor in the Pathology and Laboratory Medicine department at Medical College of Wisconsin
Katherine L. Vaidy MD Assistant Professor in the Pediatrics department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
ChildClass I Phosphatidylinositol 3-Kinases
Female
Humans
Hypergammaglobulinemia
Male
Recurrence
