In daily practice, the common occurrence of limb and/or skin necrosis, which we call “acute peripheral and/or cutaneous ischemic syndrome” (APCIS), can be a manifestation of a variety of underlying diseases, or it can be a clinical phenomenon whose etiology remains unknown despite extensive investigation. Vessel wall anomalies, embolic processes, local thrombotic injuries, dysproteinemias, or venous limb gangrene are all causes underlying the development of APCIS. Researchers provide five example instances of APCIS with various pathogenetic pathways, highlighting several clinical diseases that induce APCIS that may be of particular interest to rheumatologists, such as antiphospholipid syndrome, primary and secondary vasculitis, and cryoproteinemias. In addition, they detail a wide range of additional causes of APCIS that are not related to rheumatology. 

They suggest a preliminary diagnostic workup and a treatment strategy based on full-dose anticoagulation and immunosuppressive medication because there are no established recommendations for APCIS.