Previously published and current case of Immune checkpoint inhibitor-related acral vasculitis. Patient characteristics

Age/GenderCancerICIOnset*Skin lesionsSystemic symptomsImmunological findingsCTCAETreatment/outcome of the IRAE
Comont et al.66/MaleUrothelial bladder cancerAnti-CTLA4 (tremelimumab)Anti-PDL1 (durvalumab)8Periungual skin necrosis of several digits of both handsNoneANAs (titer 5200, speckled pattern)3ICI discontinuation; Prednisone Complete resolution of IRAE
Padda er al.52/MaleMelanomaAnti-CTLA4 (ipilimumab)4Subungual necrosis on several upper and lower limb digits, rashMyalgias, arthralgias, vision changes, jaw pain, interstitial pneumoniaNone3ICI discontinuation; Methylprednisolone, prednisone, calcium channel blockers, nitropaste, poprostenol, botulinum toxin, sildenafil, rituximabWorsening of the IRAE requiring surgical amputation of multiple distal digits
Leburel et al.60/MaleMelanomaAnti-PDL1 (UKN)BRAF and MEK inhibitors (UKN)8Necrosis of 3 fingers and the heelsArthralgia, dry mouth, paresthaesia of the feet and interstitial pneumoniaANAs (titer 160, speckled pattern), Anti-SSA AbsType III cryoglobulinemia3ICI discontinued; prednisone, calcium channel blockers, iloprost and acetylsalicylic acidPartial resolution of th.e IRAE
Gambichler et al.60/MaleMelanomaAnti-PD1 (nivolumab)Anti- CTLA4 (ipilimumab)3Subungual necrosis on the fingertips of both hands, severe gangreneNoneNone4ICI discontinued after a second course of nivolumab;Prostacyclin, methylprednisolone Worsening of the IRAE requiring surgical amputation of multiple distal digitsProgression of metastatic disease to multi-organ failure, leading to the death of the patient

*Weeks between initiation of immunotherapy and the diagnosis of vasculitis

Ab antibodies, ANA antinuclear antibody, CTCAE Common Terminology Criteria for Adverse Events, ICI immune checkpoint inhibitor, IRAE immune-related adverse event, UKN unknown