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SAT-LB88 Recurrent Dysphagia Due to an Esophageal Stricture in a Patient With Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED)

Background: Chronic Mucocutaneous Candidiasis is one of the hallmark features of Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED). Untreated esophageal candidiasis may lead to complications such as esophageal stricture, rupture and or fistula formation(1,2). Case Presentation:...

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Autores principales: Johnson, Timothy, Gabriely, Ilan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7208786/
http://dx.doi.org/10.1210/jendso/bvaa046.2204
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author Johnson, Timothy
Gabriely, Ilan
author_facet Johnson, Timothy
Gabriely, Ilan
author_sort Johnson, Timothy
collection PubMed
description Background: Chronic Mucocutaneous Candidiasis is one of the hallmark features of Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED). Untreated esophageal candidiasis may lead to complications such as esophageal stricture, rupture and or fistula formation(1,2). Case Presentation:A 31-year-old man with a past medical history significant for APECED with involvement including hypoparathyroidism, hypothyroidism and adrenal insufficiency presented to the Emergency Department with dysphagia and globus sensation after eating. This was the fourth time in the last 2 years that the patient had presented with this chief complaint. The patient underwent emergent esophagogastroduodenoscopy (EGD) where a large meat bolus was identified at approximately 20 cm and removed. A discrete esophageal tear at 19-20 cm was identified and clipped. The esophagus was noted to be diffusely narrowed with mild corrugation, and biopsies showed evidence of esophageal candidiasis. A chest CT scan demonstrated diffuse pneumomediastinum and soft tissue emphysema throughout the esophagus, suspicious for a tear. Fluoroscopic Esophagogram showed mild narrowing of the upper third of the esophagus without evidence of gross extravasation. The patient was placed on NPO (nothing by mouth) diet, started total parenteral nutrition, and treated with IV Fluconazole, administered for 10 days and then transitioned to oral Fluconazole for a total of 21 days, with improvement. A subsequent repeat fluoroscopic esophagogram showed absence of extravasation at the previously identified level of the esophagus. The patient is currently scheduled for a repeat EGD in 3 months. Conclusion:This case demonstrates a significant complication of APECED involving esophageal candidiasis. Timely diagnosis and treatment of esophageal candidiasis in APECED may prevent life-threatening esophageal pathology. References:1)Kim, Byung-Wook et al. Esophagomediastinal fistula and esophageal stricture as a complication of esophageal candidiasis: A case report. Gastrointestinal Endoscopy, Volume 52, Issue 6, 772 – 775.2)Hyun J, Chun H, Keum B et al. Candida esophagitis complicated by esophageal stricture. Endoscopy 2010; 42(S02): 180 - 181. doi:10.1055/s-0029-1244014.
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spelling pubmed-72087862020-05-13 SAT-LB88 Recurrent Dysphagia Due to an Esophageal Stricture in a Patient With Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED) Johnson, Timothy Gabriely, Ilan J Endocr Soc Thyroid Background: Chronic Mucocutaneous Candidiasis is one of the hallmark features of Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED). Untreated esophageal candidiasis may lead to complications such as esophageal stricture, rupture and or fistula formation(1,2). Case Presentation:A 31-year-old man with a past medical history significant for APECED with involvement including hypoparathyroidism, hypothyroidism and adrenal insufficiency presented to the Emergency Department with dysphagia and globus sensation after eating. This was the fourth time in the last 2 years that the patient had presented with this chief complaint. The patient underwent emergent esophagogastroduodenoscopy (EGD) where a large meat bolus was identified at approximately 20 cm and removed. A discrete esophageal tear at 19-20 cm was identified and clipped. The esophagus was noted to be diffusely narrowed with mild corrugation, and biopsies showed evidence of esophageal candidiasis. A chest CT scan demonstrated diffuse pneumomediastinum and soft tissue emphysema throughout the esophagus, suspicious for a tear. Fluoroscopic Esophagogram showed mild narrowing of the upper third of the esophagus without evidence of gross extravasation. The patient was placed on NPO (nothing by mouth) diet, started total parenteral nutrition, and treated with IV Fluconazole, administered for 10 days and then transitioned to oral Fluconazole for a total of 21 days, with improvement. A subsequent repeat fluoroscopic esophagogram showed absence of extravasation at the previously identified level of the esophagus. The patient is currently scheduled for a repeat EGD in 3 months. Conclusion:This case demonstrates a significant complication of APECED involving esophageal candidiasis. Timely diagnosis and treatment of esophageal candidiasis in APECED may prevent life-threatening esophageal pathology. References:1)Kim, Byung-Wook et al. Esophagomediastinal fistula and esophageal stricture as a complication of esophageal candidiasis: A case report. Gastrointestinal Endoscopy, Volume 52, Issue 6, 772 – 775.2)Hyun J, Chun H, Keum B et al. Candida esophagitis complicated by esophageal stricture. Endoscopy 2010; 42(S02): 180 - 181. doi:10.1055/s-0029-1244014. Oxford University Press 2020-05-08 /pmc/articles/PMC7208786/ http://dx.doi.org/10.1210/jendso/bvaa046.2204 Text en © Endocrine Society 2020. http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (http://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Thyroid
Johnson, Timothy
Gabriely, Ilan
SAT-LB88 Recurrent Dysphagia Due to an Esophageal Stricture in a Patient With Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED)
title SAT-LB88 Recurrent Dysphagia Due to an Esophageal Stricture in a Patient With Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED)
title_full SAT-LB88 Recurrent Dysphagia Due to an Esophageal Stricture in a Patient With Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED)
title_fullStr SAT-LB88 Recurrent Dysphagia Due to an Esophageal Stricture in a Patient With Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED)
title_full_unstemmed SAT-LB88 Recurrent Dysphagia Due to an Esophageal Stricture in a Patient With Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED)
title_short SAT-LB88 Recurrent Dysphagia Due to an Esophageal Stricture in a Patient With Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy (APECED)
title_sort sat-lb88 recurrent dysphagia due to an esophageal stricture in a patient with autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (apeced)
topic Thyroid
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7208786/
http://dx.doi.org/10.1210/jendso/bvaa046.2204
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