Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome Manifesting as Lymphocytic Interstitial Pneumonia and Treatment-Resistant Bullous Pemphigoid

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 ly...

Ausführliche Beschreibung

Gespeichert in:

Bibliographische Detailangaben
Veröffentlicht in:	Pediatric allergy, immunology, and pulmonology immunology, and pulmonology, 2021-06, Vol.34 (2), p.76-79
Hauptverfasser:	Andersen, Stine Maria, Fage, Simon, Rubak, Sune Leisgaard, Holm, Mette, Jensen, Jens Magnus Bernth, Mogensen, Trine, Deleuran, Mette
Format:	Artikel
Sprache:	eng
Schlagworte:	Allergy Autoimmune diseases Bullous pemphigoid Case Reports Child Diabetes Mellitus, Type 1 - congenital Diarrhea Forkhead Transcription Factors Genetic Diseases, X-Linked Health risks Humans Immune System Diseases - congenital Immunology Immunoregulation Life Sciences & Biomedicine Lung Diseases, Interstitial - diagnosis Lung Diseases, Interstitial - genetics Lymphocytes Lymphocytes T Male Pediatrics Pemphigoid, Bullous - diagnosis Pemphigoid, Bullous - drug therapy Pemphigoid, Bullous - genetics Pneumonia Respiratory System Science & Technology T-Lymphocytes, Regulatory
Online-Zugang:	Volltext
Tags:	Tag hinzufügen Keine Tags, Fügen Sie den ersten Tag hinzu!

Beschreibung
Zusammenfassung:	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.
ISSN:	2151-321X 2151-3228
DOI:	10.1089/ped.2020.1307