Disease directory
Juvenile xanthogranuloma
Published: September 18, 2026Updated: September 18, 2026
Important: Informational content intended for healthcare professionals. It does not replace clinical evaluation or establish a doctor-patient relationship.
Overview
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Names : Juvenile xanthogranuloma (JXG); belongs to the group of non-Langerhans histiocytosis (NLCH).
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First description : Adamson, 1905 (“congenital xanthoma multiplex”); the modern nomenclature was consolidated in the mid-twentieth century.
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Epidemiology : Predominant in infants and young children; typically first appears within the first two years of life; may be congenital. Typical benign cutaneous course with spontaneous involution.
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Current classification : Included in the histiocytosis family (group C/NLCH) and in histiocytic and dendritic tumors according to the Histiocyte Society/WHO review.
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Codes : ICD-10 : D76.3 (other histiocytoses); ICD-11 : 2B31.0 (Juvenile xanthogranuloma).
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