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Hidradenitis Suppurativa as a Potential Subtype of Autoinflammatory Keratinization Disease

Frontiers in immunology, 2020-05, Vol.11, p.847-847 [Peer Reviewed Journal]

Copyright © 2020 Nomura. ;COPYRIGHT 2020 Frontiers Research Foundation ;Copyright © 2020 Nomura. 2020 Nomura ;ISSN: 1664-3224 ;EISSN: 1664-3224 ;DOI: 10.3389/fimmu.2020.00847 ;PMID: 32508815

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Title:
Hidradenitis Suppurativa as a Potential Subtype of Autoinflammatory Keratinization Disease
Author: Nomura, Toshifumi
Subjects: Amyloid Precursor Protein Secretases - genetics ; Animals ; autoinflammatory keratinization disease ; Biological Products - therapeutic use ; hidradenitis suppurativa ; Hidradenitis Suppurativa - genetics ; Hidradenitis Suppurativa - immunology ; Hidradenitis Suppurativa - therapy ; Humans ; Immunology ; Immunotherapy - methods ; interleukin 17 ; interleukin 1β ; Interleukin-17 - metabolism ; Interleukin-1beta - metabolism ; Loss of Function Mutation ; Mice ; Monoclonal antibodies ; Notch signaling ; Receptors, Notch - genetics ; Receptors, Notch - metabolism ; T-Lymphocytes, Regulatory - immunology ; Th17 Cells - immunology ; γ-secretase
Is Part Of: Frontiers in immunology, 2020-05, Vol.11, p.847-847
Description: Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition, clinically characterized by boiled cysts, comedones, abscesses, hypertrophic scars, and/or sinus tracts typically in the apocrine-gland-rich areas such as the axillae, groin, and/or buttocks. Although its precise pathogenic mechanisms remain unknown, I herein emphasize the importance of the following three recent discoveries in the pathogenesis of HS: First, heterozygous loss-of-function mutations in the genes encoding γ-secretase, including , and , have been identified in some patients with HS. Such genetic alterations result in hyperkeratosis, dysregulated hair follicle differentiation, and cyst formation via aberrant Notch signaling. Furthermore, / -, -, +/-, and / mice share common phenotypes of human HS, suggesting a role of aberrant keratinization in the development of HS. Second, upregulation of interleukin 1β, interleukin-36, caspase-1, and NLRP3 and dysregulation of the Th17:Treg cell axis have been demonstrated in HS samples, suggesting that autoinflammation is a key event in the pathophysiology of the disease. Notably, HS may be complicated with other autoinflammatory diseases such as inflammatory bowel diseases and pyoderma gangrenosum, again highlighting the importance of autoinflammation in HS. Last, biologics such as adalimumab, infliximab, anakinra, ustekinumab, and secukinumab are reportedly effective for moderate-to-severe HS. These findings collectively suggest that HS is closely linked with aberrant keratinization and autoinflammation, raising the question whether it represents an autoinflammatory keratinization disease, a recently proposed disease entity. In this mini review, I introduce the concept of autoinflammatory keratinization disease and attempt to address this clinically important question.
Publisher: Switzerland: Frontiers Research Foundation
Language: English
Identifier: ISSN: 1664-3224
EISSN: 1664-3224
DOI: 10.3389/fimmu.2020.00847
PMID: 32508815
Source: DOAJ Open Access Journals
GFMER Free Medical Journals
MEDLINE
PubMed Central
ROAD: Directory of Open Access Scholarly Resources

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Hidradenitis Suppurativa as a Potential Subtype of Autoinflammatory Keratinization Disease

Copyright © 2020 Nomura. ;COPYRIGHT 2020 Frontiers Research Foundation ;Copyright © 2020 Nomura. 2020 Nomura ;ISSN: 1664-3224 ;EISSN: 1664-3224 ;DOI: 10.3389/fimmu.2020.00847 ;PMID: 32508815

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