Hidradenitis suppurativa (HS) is a chronic inflammatory disease usually involving the major skin folds characterized by a multifactorial pathogenesis and a wide spectrum of clinical manifestations. It can also rarely present in association with other diseases as complex clinical syndromes, causing additional diagnostic and therapeutic challenges. Different etiopathologic factors contribute to follicular inflammation and suppurative lesions of syndromic HS, including follicular hyperkeratinization and plugging, as well as activation of autoinflammatory pathways. Patients with syndromic HS frequently have a severe disease course, presenting with atypical skin involvement, signs of systemic inflammation, and resistance to conventional treatments. Systematic classification of syndromic HS is based on clinical, pathogenetic, and genetic factors, but it is constantly evolving due to increased disease awareness. Treatment of syndromic HS is difficult and should be personalized on a case-by-case basis. Investigating syndromic HS can lead to useful insights on genetics and pathogenesis, translating into new clinical approaches for sporadic hidradenitis. We review the classification, clinical presentation, disease associations, and therapeutic management of syndromic HS, focusing mainly on its autoinflammatory syndromes PASH, PAPASH, PsAPASH, and PASS.

PASH, PAPASH, PsAPASH, and PASS: The autoinflammatory syndromes of hidradenitis suppurativa / S. Garcovich, G. Genovese, C. Moltrasio, D. Malvaso, A.V. Marzano. - In: CLINICS IN DERMATOLOGY. - ISSN 0738-081X. - (2020). [Epub ahead of print] [10.1016/j.clindermatol.2020.10.016]

PASH, PAPASH, PsAPASH, and PASS: The autoinflammatory syndromes of hidradenitis suppurativa

G. Genovese
Secondo
;
A.V. Marzano
Ultimo
2020

Abstract

Hidradenitis suppurativa (HS) is a chronic inflammatory disease usually involving the major skin folds characterized by a multifactorial pathogenesis and a wide spectrum of clinical manifestations. It can also rarely present in association with other diseases as complex clinical syndromes, causing additional diagnostic and therapeutic challenges. Different etiopathologic factors contribute to follicular inflammation and suppurative lesions of syndromic HS, including follicular hyperkeratinization and plugging, as well as activation of autoinflammatory pathways. Patients with syndromic HS frequently have a severe disease course, presenting with atypical skin involvement, signs of systemic inflammation, and resistance to conventional treatments. Systematic classification of syndromic HS is based on clinical, pathogenetic, and genetic factors, but it is constantly evolving due to increased disease awareness. Treatment of syndromic HS is difficult and should be personalized on a case-by-case basis. Investigating syndromic HS can lead to useful insights on genetics and pathogenesis, translating into new clinical approaches for sporadic hidradenitis. We review the classification, clinical presentation, disease associations, and therapeutic management of syndromic HS, focusing mainly on its autoinflammatory syndromes PASH, PAPASH, PsAPASH, and PASS.
Settore MED/35 - Malattie Cutanee e Veneree
2020
16-ott-2020
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/804878
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