Hidradenitis Suppurativa as a Potential Subtype of Autoinflammatory Keratinization Disease.

Frontiers in Immunology
Toshifumi Nomura

Abstract

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 NCSTN, PSENEN, and PSEN1, 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, Psen1-/Psen2-, Psen1-, Ncstn+/-, and Notch1-/Notch2- 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 inflammato...Continue Reading

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Citations

Nov 27, 2020·International Journal of Molecular Sciences·Rossella GrattonSergio Crovella
Feb 13, 2021·Clinical and Experimental Dermatology·U İlgenS Vural
Jun 13, 2021·Actas dermo-sifiliográficas·A Peña-RosadoJ Romaní
Aug 22, 2021·Experimental Dermatology·Daniel G W JohnstonDesmond J Tobin

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BETA
biopsy

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