Juvenile Xanthogranulomatosis in a Hemophilic Boy: Case Report

Abstract

Juvenile Xanthogranulomatosis (JXG) is a condition, characterized by a proliferation of histiocytes, primarily observed in infants and young children. Cutaneous manifestations appear as yellow-orange-brown papules or nodules, typically localized on the face, neck, and upper chest. While most lesions regress spontaneously, some may require intervention for aesthetic, diagnostic, or hemorrhagic reasons. A rare case of disseminated JXG in a child with hemophilia has been reported. In this patient with severe hemophilia A, cutaneous nodules appeared, some associated with bleeding requiring appropriate management. Treatment included the administration of factor VIII to prevent bleeding during surgical procedures and secondary prophylaxis, to control recurrent bleeding. The outcome was favorable with the disappearance of the cutaneous lesions without sequelae, under regular surveillance for both medical conditions. This case highlights the rare association between juvenile xanthogranulomatosis (JXG) and hemophilia, a combination that has never been documented in the medical literature. This association only impacts the management of JXG when the cutaneous lesions bleed and their excision becomes necessary.