Introduction: Fournier's gangrene is an infective necrotizing fasciitis of external genital and perineal region. Hematologic malignancies and immunocompromised status are predisposing factors. Simultaneous occurrence of Fournier's gangrene and congenital leukemia in neonates is extremely rare.
Case presentation: We present a case of Fournier's gangrene in a 4-day-old female infant with a necrotic lesion in perineum and no history of trauma or other predisposing condition. Focusing on high blast percentage in blood cell count she was affected by acute myeloid leukemia (M4 type). Pseudomonas aeruginosa was isolated from the blood and wound culture. She was treated with broad spectrum antibiotics and supportive care. The parents refused chemotherapy and the patient was discharged from hospital. Bleeding and DIC was the cause of death in a local hospital few days later.
Conclusions: High index of suspicion is essential for diagnosis and appropriate treatment. Congenital leukemia should be considered in the differential diagnosis of a newborn with clinical features of sepsis and necrotizing fasciitis.
Keywords: Acute Myeloid Leukemia; Fournier’s Gangrene; Neonate.