PYODERMA-GANGRENOSUM ASSOCIATED WITH HEMOPHAGOCYTIC SYNDROME

Pyoderma gangrenosum is an immunopathologically evolving ulcerative dermatitis and is frequently associated with systemic diseases. We present the case of a lethal pyoderma gangrenosum associated with hemophagocytic syndrome. To our knowledge, this is the second case described in the world literature. Hemophagocytic syndrome is a rare disease, in which ingestion of red blood cells by activated macrophages occurs. The underlying immune dysregulation might be the sequela of an infection, immunosuppressive therapy or a neoplastic disease.