Amyloidosis with recurrent oral and peptic ulcers mimicking Behcet's syndrome: A case report

Abstract Introduction Amyloidosis and Behcet's syndrome (BS) are systemic diseases that affect multiple systems. Similar mucocutaneous damage occurs in amyloidosis and BS, thus causing a delayed or incorrect diagnosis. The prognosis of patients with amyloidosis is poor. Therefore, exclusion of...

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Main Authors: Jiajing Peng, Tian Liu
Format: Article
Language:English
Published: Wiley 2022-03-01
Series:Rheumatology & Autoimmunity
Subjects:
Online Access:https://doi.org/10.1002/rai2.12034
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author Jiajing Peng
Tian Liu
author_facet Jiajing Peng
Tian Liu
author_sort Jiajing Peng
collection DOAJ
description Abstract Introduction Amyloidosis and Behcet's syndrome (BS) are systemic diseases that affect multiple systems. Similar mucocutaneous damage occurs in amyloidosis and BS, thus causing a delayed or incorrect diagnosis. The prognosis of patients with amyloidosis is poor. Therefore, exclusion of other diagnoses is important before confirming the presence of BS. Case Report We report a case of a 43‐year‐old woman who initially presented with peptic ulcers, recurrent oral ulcers, and suspicious genital ulcers resembling BS. She was treated with systemic steroids at the local hospital for approximately 2 years. However, her symptoms were not relieved, and the gradual presence of macroglossia, hoarseness, periorbital purpura, proteinuria, and cardiac involvement strongly suggested amyloidosis. She was diagnosed with immunoglobulin light chain amyloidosis (lambda type) after extensive examinations, including an elevated monoclonal protein concentration, Congo red staining, and immunohistochemical staining of abdominal fat. She was then treated with a chemotherapy regimen (melphalan and dexamethasone). Conclusion Recurrent oral ulcers can be the first manifestation of amyloidosis. The diagnosis of BS requires the exclusion of other diseases, and amyloidosis should be considered as a differential diagnosis.
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spelling doaj.art-9030003ed064496397ebee66933d7ca72022-12-22T03:12:47ZengWileyRheumatology & Autoimmunity2767-14292022-03-0121404410.1002/rai2.12034Amyloidosis with recurrent oral and peptic ulcers mimicking Behcet's syndrome: A case reportJiajing Peng0Tian Liu1Department of Rheumatology and Immunology Peking University People's Hospital Beijing ChinaDepartment of Rheumatology and Immunology Peking University People's Hospital Beijing ChinaAbstract Introduction Amyloidosis and Behcet's syndrome (BS) are systemic diseases that affect multiple systems. Similar mucocutaneous damage occurs in amyloidosis and BS, thus causing a delayed or incorrect diagnosis. The prognosis of patients with amyloidosis is poor. Therefore, exclusion of other diagnoses is important before confirming the presence of BS. Case Report We report a case of a 43‐year‐old woman who initially presented with peptic ulcers, recurrent oral ulcers, and suspicious genital ulcers resembling BS. She was treated with systemic steroids at the local hospital for approximately 2 years. However, her symptoms were not relieved, and the gradual presence of macroglossia, hoarseness, periorbital purpura, proteinuria, and cardiac involvement strongly suggested amyloidosis. She was diagnosed with immunoglobulin light chain amyloidosis (lambda type) after extensive examinations, including an elevated monoclonal protein concentration, Congo red staining, and immunohistochemical staining of abdominal fat. She was then treated with a chemotherapy regimen (melphalan and dexamethasone). Conclusion Recurrent oral ulcers can be the first manifestation of amyloidosis. The diagnosis of BS requires the exclusion of other diseases, and amyloidosis should be considered as a differential diagnosis.https://doi.org/10.1002/rai2.12034amyloidosisBehcet's syndromeulcer
spellingShingle Jiajing Peng
Tian Liu
Amyloidosis with recurrent oral and peptic ulcers mimicking Behcet's syndrome: A case report
Rheumatology & Autoimmunity
amyloidosis
Behcet's syndrome
ulcer
title Amyloidosis with recurrent oral and peptic ulcers mimicking Behcet's syndrome: A case report
title_full Amyloidosis with recurrent oral and peptic ulcers mimicking Behcet's syndrome: A case report
title_fullStr Amyloidosis with recurrent oral and peptic ulcers mimicking Behcet's syndrome: A case report
title_full_unstemmed Amyloidosis with recurrent oral and peptic ulcers mimicking Behcet's syndrome: A case report
title_short Amyloidosis with recurrent oral and peptic ulcers mimicking Behcet's syndrome: A case report
title_sort amyloidosis with recurrent oral and peptic ulcers mimicking behcet s syndrome a case report
topic amyloidosis
Behcet's syndrome
ulcer
url https://doi.org/10.1002/rai2.12034
work_keys_str_mv AT jiajingpeng amyloidosiswithrecurrentoralandpepticulcersmimickingbehcetssyndromeacasereport
AT tianliu amyloidosiswithrecurrentoralandpepticulcersmimickingbehcetssyndromeacasereport