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Migratory Panniculitis with Autoimmune Cholangitis and Pancreatitis (IgG4- Related Disease): A Rare Presentation

A 30-year-old woman presented with an acute-onset high-grade fever (103°F), multiple episodes of throbbing abdominal pain in the right quadrant, and pustular red lesions on the left leg. The radiological findings were suggestive of autoimmune cholangitis and pancreatitis. The skin lesions recurred o...

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Autores principales: Malkani, Ram H, Nagral, Aabha, Karmakar, Suman, Setia, Maninder Singh
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9644787/
https://www.ncbi.nlm.nih.gov/pubmed/36386064
http://dx.doi.org/10.4103/ijd.IJD_373_20
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author Malkani, Ram H
Nagral, Aabha
Karmakar, Suman
Setia, Maninder Singh
author_facet Malkani, Ram H
Nagral, Aabha
Karmakar, Suman
Setia, Maninder Singh
author_sort Malkani, Ram H
collection PubMed
description A 30-year-old woman presented with an acute-onset high-grade fever (103°F), multiple episodes of throbbing abdominal pain in the right quadrant, and pustular red lesions on the left leg. The radiological findings were suggestive of autoimmune cholangitis and pancreatitis. The skin lesions recurred on different portions of the body, and histopathological findings showed lobular and septal lymphohistiocytic infiltrate without vasculitis. The epidermis had focal ulcerations, and the dermis showed the presence of subcutaneous fat and an infiltrate of lymphocytes, histiocytes, and neutrophils (described as lobular and septal panniculitis without vasculitis). Based on the clinical and histopathological features, a diagnosis of migratory panniculitis was made. The patient was managed with analgesics, antibiotics, and a long course of steroids; the patient responded well to these medications. To the best of our knowledge, this is one of the first case reports of migratory panniculitis associated with IgG4-related disease. Patients with migratory panniculitis should be investigated for the presence of IgG4-related autoimmune disease. However, steroids remain the drug of choice when these conditions occur together.
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spelling pubmed-96447872022-11-15 Migratory Panniculitis with Autoimmune Cholangitis and Pancreatitis (IgG4- Related Disease): A Rare Presentation Malkani, Ram H Nagral, Aabha Karmakar, Suman Setia, Maninder Singh Indian J Dermatol Case Report A 30-year-old woman presented with an acute-onset high-grade fever (103°F), multiple episodes of throbbing abdominal pain in the right quadrant, and pustular red lesions on the left leg. The radiological findings were suggestive of autoimmune cholangitis and pancreatitis. The skin lesions recurred on different portions of the body, and histopathological findings showed lobular and septal lymphohistiocytic infiltrate without vasculitis. The epidermis had focal ulcerations, and the dermis showed the presence of subcutaneous fat and an infiltrate of lymphocytes, histiocytes, and neutrophils (described as lobular and septal panniculitis without vasculitis). Based on the clinical and histopathological features, a diagnosis of migratory panniculitis was made. The patient was managed with analgesics, antibiotics, and a long course of steroids; the patient responded well to these medications. To the best of our knowledge, this is one of the first case reports of migratory panniculitis associated with IgG4-related disease. Patients with migratory panniculitis should be investigated for the presence of IgG4-related autoimmune disease. However, steroids remain the drug of choice when these conditions occur together. Wolters Kluwer - Medknow 2022 /pmc/articles/PMC9644787/ /pubmed/36386064 http://dx.doi.org/10.4103/ijd.IJD_373_20 Text en Copyright: © 2022 Indian Journal of Dermatology https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Malkani, Ram H
Nagral, Aabha
Karmakar, Suman
Setia, Maninder Singh
Migratory Panniculitis with Autoimmune Cholangitis and Pancreatitis (IgG4- Related Disease): A Rare Presentation
title Migratory Panniculitis with Autoimmune Cholangitis and Pancreatitis (IgG4- Related Disease): A Rare Presentation
title_full Migratory Panniculitis with Autoimmune Cholangitis and Pancreatitis (IgG4- Related Disease): A Rare Presentation
title_fullStr Migratory Panniculitis with Autoimmune Cholangitis and Pancreatitis (IgG4- Related Disease): A Rare Presentation
title_full_unstemmed Migratory Panniculitis with Autoimmune Cholangitis and Pancreatitis (IgG4- Related Disease): A Rare Presentation
title_short Migratory Panniculitis with Autoimmune Cholangitis and Pancreatitis (IgG4- Related Disease): A Rare Presentation
title_sort migratory panniculitis with autoimmune cholangitis and pancreatitis (igg4- related disease): a rare presentation
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9644787/
https://www.ncbi.nlm.nih.gov/pubmed/36386064
http://dx.doi.org/10.4103/ijd.IJD_373_20
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