Skin-Limited IgA Vasculitis: A Unique Presentation in an Adult Without Renal Involvement

We describe a rare case of skin-limited immunoglobulin A vasculitis (IgAV) in an adult with polycythemia vera (PV), a myeloproliferative neoplasm more commonly associated with IgA nephropathy (IgAN) than IgAV. Diagnosis was confirmed by biopsy showing IgA-dominant leukocytoclastic vasculitis without...

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Main Authors: Christian Cook, Jiwoo Kim, Escher Howard-Williams, Evan Raff
Format: Article
Language:English
Published: American College of Physicians 2025-07-01
Series:Annals of Internal Medicine: Clinical Cases
Online Access:https://www.acpjournals.org/doi/10.7326/aimcc.2024.1200
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author Christian Cook
Jiwoo Kim
Escher Howard-Williams
Evan Raff
author_facet Christian Cook
Jiwoo Kim
Escher Howard-Williams
Evan Raff
author_sort Christian Cook
collection DOAJ
description We describe a rare case of skin-limited immunoglobulin A vasculitis (IgAV) in an adult with polycythemia vera (PV), a myeloproliferative neoplasm more commonly associated with IgA nephropathy (IgAN) than IgAV. Diagnosis was confirmed by biopsy showing IgA-dominant leukocytoclastic vasculitis without systemic involvement. Our patient was treated supportively for IgAV and with ruxolitinib for PV, with resolution of skin findings and no systemic progression. Although vasculitis is more commonly associated with myelodysplastic syndromes, its occurrence with PV is rare. Careful evaluation for renal involvement in IgAV is essential to guide management and to monitor for systemic progression, particularly renal manifestations.
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series Annals of Internal Medicine: Clinical Cases
spelling doaj-art-2d7d08e81ade4c5fbd6f2f36d828a2a32025-08-20T03:50:48ZengAmerican College of PhysiciansAnnals of Internal Medicine: Clinical Cases2767-76642025-07-014710.7326/aimcc.2024.1200Skin-Limited IgA Vasculitis: A Unique Presentation in an Adult Without Renal InvolvementChristian Cook0Jiwoo Kim1Escher Howard-Williams2Evan Raff31University of North Carolina, School of Medicine, Chapel Hill, North Carolina1University of North Carolina, School of Medicine, Chapel Hill, North Carolina1University of North Carolina, School of Medicine, Chapel Hill, North Carolina1University of North Carolina, School of Medicine, Chapel Hill, North CarolinaWe describe a rare case of skin-limited immunoglobulin A vasculitis (IgAV) in an adult with polycythemia vera (PV), a myeloproliferative neoplasm more commonly associated with IgA nephropathy (IgAN) than IgAV. Diagnosis was confirmed by biopsy showing IgA-dominant leukocytoclastic vasculitis without systemic involvement. Our patient was treated supportively for IgAV and with ruxolitinib for PV, with resolution of skin findings and no systemic progression. Although vasculitis is more commonly associated with myelodysplastic syndromes, its occurrence with PV is rare. Careful evaluation for renal involvement in IgAV is essential to guide management and to monitor for systemic progression, particularly renal manifestations.https://www.acpjournals.org/doi/10.7326/aimcc.2024.1200
spellingShingle Christian Cook
Jiwoo Kim
Escher Howard-Williams
Evan Raff
Skin-Limited IgA Vasculitis: A Unique Presentation in an Adult Without Renal Involvement
Annals of Internal Medicine: Clinical Cases
title Skin-Limited IgA Vasculitis: A Unique Presentation in an Adult Without Renal Involvement
title_full Skin-Limited IgA Vasculitis: A Unique Presentation in an Adult Without Renal Involvement
title_fullStr Skin-Limited IgA Vasculitis: A Unique Presentation in an Adult Without Renal Involvement
title_full_unstemmed Skin-Limited IgA Vasculitis: A Unique Presentation in an Adult Without Renal Involvement
title_short Skin-Limited IgA Vasculitis: A Unique Presentation in an Adult Without Renal Involvement
title_sort skin limited iga vasculitis a unique presentation in an adult without renal involvement
url https://www.acpjournals.org/doi/10.7326/aimcc.2024.1200
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