Hidradenitis Secondary to Nirogacestat, a Recently Approved Desmoid Tumor Medication

    December 2024 in “ JAAD Case Reports
    Travis Frantz, David S Kirwin, Catherine Brahe
    TLDR Nirogacestat can cause severe skin issues like hidradenitis suppurativa.
    This document discusses a case of hidradenitis suppurativa (HS) developing in a 29-year-old man after starting treatment with nirogacestat, a gamma secretase (GS) inhibitor recently approved for desmoid tumors. The patient, with no prior skin conditions, experienced worsening HS symptoms over 10 months, progressing to Hurley stage 3, characterized by sinus tracts and scarring. Nirogacestat's inhibition of GS, crucial for pilosebaceous unit maintenance, is linked to HS development. Previous studies reported cutaneous toxicity in patients using GS inhibitors, highlighting the need for clinicians to recognize this potential adverse effect.
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