Systemic juvenile xanthogranuloma (JXG) is a rare histiocytic disorder. When it extends to involve two or more visceral organs alongside cutaneous or subcutaneous nodules, it defines a more severe and clinically demanding presentation that warrants a structured treatment approach.
Systemic JXG with involvement of 2 or more visceral organs — a rare presentation of this histiocytic disorder in which internal organ involvement accompanies the characteristic nodular skin findings. This multi-organ pattern shapes the choice and intensity of treatment.
Management in this setting may include chemotherapy-based strategies; for cases that are refractory to initial treatment, specific chemotherapy combinations — including cytarabine-based options — have been described.
Systemic JXG is a rare histiocytic disorder that usually manifests as multiple cutaneous and/or subcutaneous nodules and involvement of 2 or more visceral organs.
Options described include surgical resection, radiation therapy and/or chemotherapy with prednisone and vinblastine regimens used in LCH, or, for refractory cases, cytarabine and 2-chlorodeoxyadenosine.
DOI: 10.1016/j.adengl.2020.10.007
View source ↗