DOCK8 deficiency patient presenting with purpura fulminans caused by group A β-hemolytic Streptococcus sepsis

Abstract

We report a male infant presenting with Purpura fulminans (PF) secondary to sepsis caused by group A β-hemolytic Streptococcus (GAS) associated with hyper-IgE syndrome due to deletion mutation in DOCK8. The patient, previously healthy, presented with clinical symptoms of fever, lethargy, hypotension with blood culture confirming GAS infection. Subsequently, he developed purpuric skin lesions on his extremities which progressed to gangrene necessitating amputation of his fingers and toes. The findings underscore the importance of considering inborn error of immunity, especially DOCK8 deficiency, in cases of infant presenting with acute infectious PF.