Evidence on Hidradenitis Suppurativa as an Autoinflammatory Skin Disease
Abstract
:1. Introduction
2. Pathogenesis of Hidradenitis Suppurativa
3. Preclinical Evidence on the Role of Autoinflammation in Hidradenitis Suppurativa
4. Clinical Evidence of the Role of Autoinflammation in Hidradenitis Suppurativa
4.1. Syndromic Hidradenitis Suppurativa
4.2. Hidradenitis Suppurativa and Monogenic Autoinflammatory Diseases
4.3. Therapeutic Agents Targeting IL-1 Family Pathways
5. Conclusions
Author Contributions
Funding
Informed Consent Statement
Conflicts of Interest
References
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Disease | Clinical Features | Involved Genes (Inheritance Pattern) | Encoded Protein | Function |
---|---|---|---|---|
PASH syndrome | Pyoderma gangrenosum, acne, hidradenitis suppurativa | NCSTN IL1RN PSTPIP1 MEFV NOD2 NLRP3 PSMB8 PSEN1/PSEN2 | Nicastrin IL1RA PSTPIP1 Pyrin NOD2 NLRP3 PSMB8 | γ-secretase, proteolysis IL-1 pathway Inflammasome, cytoskeleton regulation Inflammasome Inflammasome, NF-κB pathway Inflammasome Proteasome complex |
PAPASH syndrome | Pyogenic arthritis, Pyoderma gangrenosum, acne, hidradenitis suppurativa | PSTPIP1 | PSTPIP1 | Inflammasome, cytoskeleton regulation |
PASS syndrome | Pyoderma gangrenosum, acne, Hidradenitis suppurativa, ankylosing spondylitis | Unknown | // | // |
PsAPASH syndrome | Psoriatic arthritis, pyoderma gangrenosum, acne, hidradenitis suppurativa | Unknown | // | // |
PsAPSASH syndrome | Pustular psoriasis, arthritis, pyoderma gangrenosum, synovitis, acne and hidradenitis suppurativa | Unknown | // | // |
SAPHO syndrome | Synovitis, acne, pustulosis, hyperostosis, osteitis | LPIN2 NOD2 PSTPIP2 IL1RN | Lipin 2 NOD2 PSTPIP1 IL1RA | Lipid biosynthesis pathway Inflammasome, NF-κB pathway Inflammasome, cytoskeleton regulation IL-1 pathway |
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D’Onghia, M.; Malvaso, D.; Galluccio, G.; Antonelli, F.; Coscarella, G.; Rubegni, P.; Peris, K.; Calabrese, L. Evidence on Hidradenitis Suppurativa as an Autoinflammatory Skin Disease. J. Clin. Med. 2024, 13, 5211. https://doi.org/10.3390/jcm13175211
D’Onghia M, Malvaso D, Galluccio G, Antonelli F, Coscarella G, Rubegni P, Peris K, Calabrese L. Evidence on Hidradenitis Suppurativa as an Autoinflammatory Skin Disease. Journal of Clinical Medicine. 2024; 13(17):5211. https://doi.org/10.3390/jcm13175211
Chicago/Turabian StyleD’Onghia, Martina, Dalma Malvaso, Giulia Galluccio, Flaminia Antonelli, Giulia Coscarella, Pietro Rubegni, Ketty Peris, and Laura Calabrese. 2024. "Evidence on Hidradenitis Suppurativa as an Autoinflammatory Skin Disease" Journal of Clinical Medicine 13, no. 17: 5211. https://doi.org/10.3390/jcm13175211