Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome Manifesting as Lymphocytic Interstitial Pneumonia and Treatment-Resistant Bullous Pemphigoid.
IPEX
bullous pemphigoid
lymphocytic interstitial pneumonia
Journal
Pediatric allergy, immunology, and pulmonology
ISSN: 2151-3228
Titre abrégé: Pediatr Allergy Immunol Pulmonol
Pays: United States
ID NLM: 101549629
Informations de publication
Date de publication:
06 2021
06 2021
Historique:
entrez:
18
6
2021
pubmed:
19
6
2021
medline:
14
1
2022
Statut:
ppublish
Résumé
Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare immune deficiency with a broad clinical presentation. IPEX syndrome causes dysfunctional regulatory T cells, increasing the risk of autoimmune diseases. In this case report, we describe a 7-year-old boy with lymphocytic interstitial pneumonia and bullous pemphigoid who was recently diagnosed with IPEX syndrome.
Identifiants
pubmed: 34143686
doi: 10.1089/ped.2020.1307
pmc: PMC8329723
doi:
Substances chimiques
Forkhead Transcription Factors
0
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
76-79Références
N Engl J Med. 2009 Oct 22;361(17):1710-3
pubmed: 19846862
Pediatr Allergy Immunol Pulmonol. 2010 Mar;23(1):69-85
pubmed: 22332032
Paediatr Respir Rev. 2011 Dec;12(4):230-7
pubmed: 22018036
J Allergy Clin Immunol. 2018 Dec;142(6):1818-1830.e6
pubmed: 29704593
Orphanet J Rare Dis. 2014 Dec 10;9:185
pubmed: 25491396
J Am Acad Dermatol. 2006 Jul;55(1):143-8
pubmed: 16781310
Arch Dermatol. 2004 Apr;140(4):466-72
pubmed: 15096376
Curr Opin Pediatr. 2013 Dec;25(6):708-14
pubmed: 24240290
Front Immunol. 2018 Nov 01;9:2411
pubmed: 30443250
Curr Opin Pediatr. 2001 Dec;13(6):533-8
pubmed: 11753102
Pediatr Pulmonol. 2020 Apr;55(4):909-917
pubmed: 32040879
Orphanet J Rare Dis. 2015 Sep 25;10:122
pubmed: 26408013
Front Immunol. 2017 Dec 08;8:1752
pubmed: 29276517
Future Healthc J. 2019 Jun;6(Suppl 2):4
pubmed: 31572905
Clin Chest Med. 2016 Sep;37(3):463-74
pubmed: 27514593
J Clin Diagn Res. 2015 Sep;9(9):OE01-5
pubmed: 26500941