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Cutaneous Deep Ulcerations as Initial Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Differential Diagnosis

Background: Granulomatosis with polyangiitis (GPA) is an antineutrophil-cytoplasmic-antibody (ANCA)-associated small-vessel vasculitis characterized by necrotizing granulomatous inflammation. Symptoms of skin involvement can appear in 30–50% of patients with GPA, and may present as the initial prese...

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Autores principales: Qian, Jiandan, Li, Jiawen, Li, Jun, Wang, Guiqiang, Zhao, Hong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10057340/
https://www.ncbi.nlm.nih.gov/pubmed/36984564
http://dx.doi.org/10.3390/medicina59030563
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author Qian, Jiandan
Li, Jiawen
Li, Jun
Wang, Guiqiang
Zhao, Hong
author_facet Qian, Jiandan
Li, Jiawen
Li, Jun
Wang, Guiqiang
Zhao, Hong
author_sort Qian, Jiandan
collection PubMed
description Background: Granulomatosis with polyangiitis (GPA) is an antineutrophil-cytoplasmic-antibody (ANCA)-associated small-vessel vasculitis characterized by necrotizing granulomatous inflammation. Symptoms of skin involvement can appear in 30–50% of patients with GPA, and may present as the initial presentation. Case Presentation: We describe two patients who presented with multiple deep, large, nonhealing skin ulcers postoperatively with purulent drainage and fever. Both patients were diagnosed with GPA after an extensive evaluation, including histopathology. Infectious, connective tissue disease and malignant etiologies were excluded. Their cANCA and PR3-ANCA levels were positive. Patient 2 was diagnosed early and recovered well after treatment with corticosteroids and rituximab; however, Patient 1 had a poor prognosis due to a long disease course. Conclusions: Diseases with multiple deep, large skin ulcers and fever can be infectious or noninfectious. Atypical manifestations may lead to missed diagnosis and misdiagnosis. GPA may initially present in a localized form before progressing to a generalized disease. The two cases we have highlighted will prompt clinicians to nevertheless call for a low threshold for diagnosis.
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spelling pubmed-100573402023-03-30 Cutaneous Deep Ulcerations as Initial Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Differential Diagnosis Qian, Jiandan Li, Jiawen Li, Jun Wang, Guiqiang Zhao, Hong Medicina (Kaunas) Case Report Background: Granulomatosis with polyangiitis (GPA) is an antineutrophil-cytoplasmic-antibody (ANCA)-associated small-vessel vasculitis characterized by necrotizing granulomatous inflammation. Symptoms of skin involvement can appear in 30–50% of patients with GPA, and may present as the initial presentation. Case Presentation: We describe two patients who presented with multiple deep, large, nonhealing skin ulcers postoperatively with purulent drainage and fever. Both patients were diagnosed with GPA after an extensive evaluation, including histopathology. Infectious, connective tissue disease and malignant etiologies were excluded. Their cANCA and PR3-ANCA levels were positive. Patient 2 was diagnosed early and recovered well after treatment with corticosteroids and rituximab; however, Patient 1 had a poor prognosis due to a long disease course. Conclusions: Diseases with multiple deep, large skin ulcers and fever can be infectious or noninfectious. Atypical manifestations may lead to missed diagnosis and misdiagnosis. GPA may initially present in a localized form before progressing to a generalized disease. The two cases we have highlighted will prompt clinicians to nevertheless call for a low threshold for diagnosis. MDPI 2023-03-14 /pmc/articles/PMC10057340/ /pubmed/36984564 http://dx.doi.org/10.3390/medicina59030563 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Qian, Jiandan
Li, Jiawen
Li, Jun
Wang, Guiqiang
Zhao, Hong
Cutaneous Deep Ulcerations as Initial Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Differential Diagnosis
title Cutaneous Deep Ulcerations as Initial Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Differential Diagnosis
title_full Cutaneous Deep Ulcerations as Initial Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Differential Diagnosis
title_fullStr Cutaneous Deep Ulcerations as Initial Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Differential Diagnosis
title_full_unstemmed Cutaneous Deep Ulcerations as Initial Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Differential Diagnosis
title_short Cutaneous Deep Ulcerations as Initial Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Differential Diagnosis
title_sort cutaneous deep ulcerations as initial presentations of granulomatosis with polyangiitis: two case reports and differential diagnosis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10057340/
https://www.ncbi.nlm.nih.gov/pubmed/36984564
http://dx.doi.org/10.3390/medicina59030563
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