K Belcadi*, Z Isfaoun, O EL-Athmani, I El-Abdallaoui, IN Ansari, M Lakhrissi, A Guindo, M El-Kababri, A Kili, L Hessissen, S Sefiani and M El-Khorassani
Published on: 15th May, 2024
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.
Micaela Redivo, María del Pilar Mingheira, Federica Fernandez Long and Carlos Santiago Ruggeri*
Published on: 23rd August, 2024
Xanthogranulomatosis is an uncommon disease affecting non-Langerhans histiocytes. Laryngeal involvement is very rare.We describe the clinical case of an adult patient with disseminated xanthogranulomas affecting the supraglottic region of the larynx, leading to thickening and immobility of the epiglottis. The patient experienced inspiratory dyspnea on exertion. A transoral supraglottic epiglottectomy was performed, and a tracheostomy was subsequently required.
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