How do you approach an older male with a chronic and persistent granulomatous cutaneous medium vessel vasculitis that has been unresponsive/worsened on high dose steroids, cyclophosphamide, and rituximab immunosuppressive agents with a thorough and unremarkable serologic, infectious, and imaging work-up?

These types of patients vex all rheumatologists at one time or another during the course of clinical practice. The primary concern that is usually raised by a rheumatic disorder unresponsive to therapy is a paraneoplastic disorder. When you say you have a “thorough imaging work-up,” what do you mean...

I'd consider a rebiopsy of the skin looking for an alternate diagnosis if unresponsive to two keystone medications of vasculitis treatment and steroids.