“…The great majority of patients with AOSD and atypical cutaneous lesions had persistent and severe disease, with a considerable frequency (23%) of clinical complications, including serositis, myopericarditis, lung involvement, abdominal pain, neurologic involvement, and reactive hemophagocytic syndrome [7,10,16–19,24,29,30,31,37] . Thus, most patients required medium or high doses of glucocorticoids (including intravenous methylprednisolone pulse therapy in some cases) and, in nearly 40%, a more potent or maintenance immunotherapy consisting of immunosuppressant drugs (including methotrexate, azathioprine, cyclosporine A, and hydroxychloroquine) [6,8–10,12,15,16,18–20,24,25,28,29,31,34,39,40] and/or biologic agents (mainly anakinra or tocilizumab) [8,10,15,21] to control or manage symptoms because they had an intermittent/polycyclic or chronic systemic course.…”