Cutaneous barrier leakage and gut inflammation drive skin disease in Omenn syndrome.

Animals Cohort Studies DNA-Binding Proteins / genetics Dermatitis / immunology Disease Models, Animal Gastrointestinal Microbiome Humans Inflammation / immunology Intestines / immunology Lymphocyte Activation Mice Mice, Inbred C57BL Receptors, CCR4 / metabolism Severe Combined Immunodeficiency / immunology Skin / pathology Th1 Cells / immunology Tight Junctions / pathology

LPS RAG T cells chemokines cytokines dysbiosis erythroderma gut-skin axis immune-mediated disease skin inflammation

Journal

The Journal of allergy and clinical immunology

ISSN: 1097-6825

Titre abrégé: J Allergy Clin Immunol

Pays: United States

ID NLM: 1275002

Informations de publication

Date de publication:
11 2020

Historique:

received: 18 09 2019

revised: 11 03 2020

accepted: 06 04 2020

pubmed: 21 4 2020

medline: 16 3 2021

entrez: 21 4 2020

Statut: ppublish

Résumé

Severe early-onset erythroderma and gut inflammation, with massive tissue infiltration of oligoclonal activated T cells are the hallmark of Omenn syndrome (OS). The impact of altered gut homeostasis in the cutaneous manifestations of OS remains to be clarified. We analyzed a cohort of 15 patients with OS and the 129Sv/C57BL/6 knock-in Rag2 We show that memory/activated T cells from patients with OS and from the Rag2 These results support the existence of an interplay between gut and skin that can sustain skin inflammation in OS.

Sections du résumé

BACKGROUND

Severe early-onset erythroderma and gut inflammation, with massive tissue infiltration of oligoclonal activated T cells are the hallmark of Omenn syndrome (OS).

OBJECTIVE

The impact of altered gut homeostasis in the cutaneous manifestations of OS remains to be clarified.

METHODS

We analyzed a cohort of 15 patients with OS and the 129Sv/C57BL/6 knock-in Rag2

RESULTS

We show that memory/activated T cells from patients with OS and from the Rag2

CONCLUSIONS

These results support the existence of an interplay between gut and skin that can sustain skin inflammation in OS.

Identifiants

DOI: 10.1016/j.jaci.2020.04.005 PMID: 32311393 PMC: PMC7649331

pubmed: 32311393

pii: S0091-6749(20)30492-9

doi: 10.1016/j.jaci.2020.04.005

pmc: PMC7649331

pii:

doi:

Substances chimiques

Ccr4 protein, mouse 0

DNA-Binding Proteins 0

Rag2 protein, mouse 0

Receptors, CCR4 0

Types de publication

Journal Article Research Support, N.I.H., Intramural Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

Pagination

1165-1179.e11

Commentaires et corrections

Type : ErratumIn

Informations de copyright

Références

JCI Insight. 2016 Jul 7;1(10):

pubmed: 27478874

PLoS One. 2014 Mar 11;9(3):e91054

pubmed: 24618819

Nat Rev Immunol. 2016 Apr;16(4):234-46

pubmed: 26996199

J Leukoc Biol. 2013 Dec;94(6):1221-30

pubmed: 24052573

J Immunol. 2006 Sep 1;177(5):3369-79

pubmed: 16920978

J Exp Med. 2016 Mar 7;213(3):355-75

pubmed: 26926994

Clin Rev Allergy Immunol. 2014 Apr;46(2):112-9

pubmed: 23760761

Front Physiol. 2012 Feb 06;3:13

pubmed: 22347192

Annu Rev Immunol. 2017 Apr 26;35:31-52

pubmed: 27860528

Nat Rev Immunol. 2014 May;14(5):289-301

pubmed: 24722477

Science. 2012 Aug 31;337(6098):1115-9

pubmed: 22837383

J Invest Dermatol. 2017 Apr;137(4):855-864

pubmed: 27887954

Drugs. 2012 Jun 18;72(9):1293-8

pubmed: 22686619

J Leukoc Biol. 2001 Oct;70(4):617-23

pubmed: 11590199

J Invest Dermatol. 2002 Aug;119(2):424-32

pubmed: 12190866

J Immunol. 2013 Feb 1;190(3):970-6

pubmed: 23255361

J Invest Dermatol. 2008 Nov;128(11):2569-2571

pubmed: 18927538

J Clin Invest. 1994 Jul;94(1):202-9

pubmed: 8040262

Curr Top Microbiol Immunol. 2006;308:83-116

pubmed: 16922087

J Cell Biol. 2006 Nov 20;175(4):661-70

pubmed: 17116754

Front Immunol. 2019 Jun 25;10:1440

pubmed: 31293591

Pediatr Dev Pathol. 2001 May-Jun;4(3):237-45

pubmed: 11370261

J Immunol. 2001 Jan 1;166(1):103-11

pubmed: 11123282

J Invest Dermatol. 2008 Apr;128(4):917-25

pubmed: 17943185

J Clin Invest. 2011 Jun;121(6):2210-20

pubmed: 21537081

Nucleic Acids Res. 2013 Jan;41(Database issue):D590-6

pubmed: 23193283

J Invest Dermatol. 2009 Nov;129(11):2552-66

pubmed: 19474804

Nature. 1999 Aug 19;400(6746):776-80

pubmed: 10466728

Eur J Immunol. 2002 Nov;32(11):3171-80

pubmed: 12555662

J Allergy Clin Immunol. 2008 Jan;121(1):129-34

pubmed: 18028995

Nat Med. 2002 Feb;8(2):157-65

pubmed: 11821900

Nat Rev Immunol. 2009 Oct;9(10):679-91

pubmed: 19763149

PLoS One. 2008 Jul 23;3(7):e2719

pubmed: 18648509

J Clin Invest. 2007 May;117(5):1260-9

pubmed: 17476358

Pediatr Crit Care Med. 2005 May;6(3 Suppl):S99-S107

pubmed: 15857568

Genome Biol. 2011 Jun 24;12(6):R60

pubmed: 21702898

Genome Res. 2013 Dec;23(12):2103-14

pubmed: 24170601

J Am Acad Dermatol. 2013 May;68(5):875-6

pubmed: 23602178

Nat Rev Gastroenterol Hepatol. 2015 Oct;12(10):561-71

pubmed: 26260366

Inflamm Bowel Dis. 2010 Nov;16(11):1969-77

pubmed: 20848507

Genome Res. 2012 May;22(5):850-9

pubmed: 22310478

Dermatology. 2006;213(4):284-92

pubmed: 17135733

J Immunol. 2008 Jan 1;180(1):122-9

pubmed: 18097011

N Engl J Med. 2006 Feb 9;354(6):610-21

pubmed: 16467548

Nature. 1997 Oct 30;389(6654):978-81

pubmed: 9353122

J Exp Med. 2001 Nov 19;194(10):1541-7

pubmed: 11714760

Clin Mol Allergy. 2008 Sep 22;6:11

pubmed: 18808715

Nat Methods. 2010 May;7(5):335-6

pubmed: 20383131

PLoS One. 2013 Jul 15;8(7):e68739

pubmed: 23869230

J Exp Med. 1991 Dec 1;174(6):1461-6

pubmed: 1720810

Semin Immunopathol. 2007 Apr;29(1):3-14

pubmed: 17621950

Nat Commun. 2015 Sep 29;6:8424

pubmed: 26416167

Nature. 2011 Aug 14;477(7363):216-9

pubmed: 21841802

Gut Microbes. 2016 Nov;7(6):503-511

pubmed: 27575988

Clin Gastroenterol Hepatol. 2008 Jul;6(7):759-64

pubmed: 18456568

J Invest Dermatol. 2001 Feb;116(2):330-8

pubmed: 11180011