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BACKGROUND Hidradenitis suppurativa (HS) is characterized by periodic worsening of both clinical manifestations and symptoms. The aim was to investigate the role of flare outbreak as a possible predictive factor of response to Adalimumab. METHODS 115 HS patients in treatment with adalimumab, with moderate-severe HS, ≥3 abscesses and inflammatory-nodules (ANs) from 5 Italian centers were included in this retrospective analysis. The information about gender, ages at onset/baseline, therapeutic delay, family history, body mass index, smoking, comorbidities, phenotypes, body areas, severity indexes at baseline was collected. Baseline characteristics, total number and timeline of flares were analysed by regression and survival analysis with Hidradenitis Suppurativa Clinical Response (HiSCR). RESULTS During the observational period, 80.9% of patients developed flares, detecting 252 flares. Univariate model identified five factors associated with the absence of response: age (p-value=0.020), comorbidities (p-value=0.030), genital-perineal involvement (pvalue= 0.004), no response at week-12 (p-value=0.027), and flares outbreak (p-value=0.010). Joint analysis of recurrent and terminal events showed a positive correlation between flare recurrence and no-response (p-value

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.