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Published on: June 15, 2020
Systemic vasculitis associated with vemurafenib treatment: Case report and literature review
Adrien Mirouse1, Léa Savey, Fanny Domont
1APHP, Service de Médecine Interne et Immunologie clinique, Groupe Hospitalier Pitié-Salpêtrière DHU Inflammation, Immunopathologie, Biothérapie, Université Pierre et Marie Curie APHP, Service de Dermatologie, Groupe Hospitalier Pitié-Salpêtrière APHP, Service de Néphrologie et Dialyse, Hôpital Tenon APHP, Service d'Anatomopathologie, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
Rationale:
Vemurafenib, an inhibitor of mutated B-rapidly accelerated fibrosarcoma, is frequently used in the treatment of melanoma and Erdheim-Chester disease (ECD) patients. Inflammatory adverse effects have been increasingly reported after vemurafenib treatment.
Patient Concerns And Diagnose:
We report 6 cases of vemurafenib-associated vasculitis, of whom a personal case of a 75-year-old man with history of ECD who developed purpura and rapidly progressive pauci-immune glomerulonephritis during treatment with vemurafenib.
Intervention:
In the 5 others cases from the literature, all patients presented skin vasculitis, and with joint involvement in 60% of them. Vemurafenib treatment was stopped (n = 3), continued at reduced doses (n = 1), or continued at the same dose (n = 2).
Outcomes:
Three patients (50%) received corticosteroids combined with cyclophosphamide (n = 1), and all achieved remission of vasculitis. One patient experienced vasculitis relapse after vemurafenib therapy was restarted.
Lessons:
Systemic vasculitis is a rare vemurafenib-associated adverse event that may be life-threatening.

