Background:   SLE in children has many manifestations. In several studies on SLE in children, gangrene and Raynaud phenomenon have been described as a rare manifestation of SLE during its course in children.
Case Report: We present the case of a 12-year-old girl referred to our center, presenting with peripheral gangrene plus Raynaud’s phenomenon, who proved to have SLE. Our patient was treated with steroids and mycophenolate mofetil. She appeared to respond to this combination judging by the disappearance of the digital cyanosis, appearance of extremity pulses, and return of renal function.
Conclusions: This case highlights the importance of precise management and awareness of very rare manifestations of a common disease like SLE. Gangrene can be initial symptom of SLE in children. We recommend SLE evolution in all children with gangrene symptom.

Keywords: systemic lupus erythematosus, Gangrene, Raynaud phenomenon, children