Background and Aims Mastocytosis consists of a heterogeneous group of disorders with diverse clinical presentations. It is characterized by an abnormal increase in tissue mast cells, which can be limited to the skin or infiltrate the bone marrow and other organs with or without skin involvement¹ Mast cell degranulation and subsequent release of vasoactive amines may occur in response to a variety of non-immune triggers leading to, as its most severe manifestation, a clinical picture of anaphylactic shock.

Methods Patient was presented in the emergency department with complaints of severe pain in the scrotal area and he was diagnosed with fournier’s gangrene. He was diagnosed with advanced systemic mastocytosis. He was on immune therapy already for this, which explains his ascites and bilateral chest infiltrate. He was hypertensive and has had issues with blood coagulation in the past as well. He was in sepsis induced DIC when presented in hospital. He had deranged coagulation factors. On auscultation systolic murmur could be heard in the aortic area. MDT discussion with urology, anesthetic & hematology consultants were done. He had general anaesthetic for the surgery and rectus sheath block followed by catheter placement. Surgeon made the plan to do laparotomy and do explanation of extensive gangrene.

Conclusions Management of patients within all categories of mastocytosis includes avoidance of factors triggering acute mediator release, treatment of acute mast cell mediator release, avoiding medical and non-medical reasons for histamine release. The Royal college of anaesthetists have given some guidelines which could come handy if such cases come in emergency².