F1000Research (Jul 2021)

Case Report: PsAPSASH syndrome: an alternative phenotype of syndromic hidradenitis suppurativa treated with the IL-17A inhibitor secukinumab [version 2; peer review: 2 approved]

  • Georgios Nikolakis,
  • Katja Kreibich,
  • Aristeidis Vaiopoulos,
  • Katarzyna Kaleta,
  • Joud Talas,
  • Markus Becker,
  • Christos C. Zouboulis

DOI
https://doi.org/10.12688/f1000research.52100.2
Journal volume & issue
Vol. 10

Abstract

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Syndromic hidradenitis suppurativa (HS) is a form of symptom constellations, which differs from the familial and genetic form and comprises predominantly osteoarticular manifestations. Many forms include pyoderma gangrenosum and acne (PASH), pyogenic arthritis (PAPASH), spondyloarthritis (PASS) and psoriatic arthritis (PsAPASH) and are categorized in the autoinflammatory syndromes. Anti-TNF-α and anti-IL-1a blockade are between the therapeutic approaches that improve skin symptoms and prevent permanent osteoarticular damage. This case report refers to the successful treatment of a mixed phenotype of the aforementioned symptoms using the IL-17A inhibitor secukinumab after initial treatment with adalimumab. The therapy improved both cutaneous and reported osteoarticular symptoms. Different approaches for these recalcitrant HS syndromes are essential in order to achieve long-term remission for those patients.