Abstract
Objective: The purpose is to discuss this rare disease that demands high clinical suspicion, showing a severe presentation, comparison with other cases in the literature, form of treatment and clinical follow up. Case detail: A 60-year old woman, previously healthy, presented livedo reticularis, cutaneous ulcer and lower limb gangrene. Received steroid treatment, without resolution. She developed sepsis with cutaneous focus, contraindicating immunossupressant. Received plasmapheresis and immunoglobulin, with stabilization of the disease. However, the lower limbs had to be amputed. This report shows a rare and severe presentation of CPAN, with rapid progression to limb lower gangrene. Initially, aggressive clinical management was necessary, with corticosteroid therapy, without response. Alternative therapy with immunoglobulin and plasmapheresis, also suitable in the context of associated sepsis was administered. Treatment was effective for clinical control; however amputation of lower limbs was inevitable. Final considerations: Treatment initially involved corticosteroid therapy, but the patient developed sepsis with a cutaneous focus and progression of ischemia. Plasmapheresis and immunoglobulin were indicated, because of their safety in the infectious scenario. The patient evolved with clinical stabilization, and was followed with immunopression with prednisone, and Azatioprine for 1 year, without reccurence.