Chronic granulomatous disease and acute neutrophilic dermatosis

Chronic granulomatous disease is an inherited disorder characterized by defective oxidative killing by neutrophils and other phagocytes. This results in susceptibility to persistent and life-threatening infections. We describe a 25-year-old man with chronic granulomatous disease who presented with an acute, febrile neutrophilic dermatosis. This indicates that normal neutrophil intracellular killing mechanisms are not essential in the pathogenesis of neutrophilic dermatoses.