Juvenile xanthogranuloma pathology

Author: Adjunct A/Prof Patrick Emanuel, Dermatopathologist, Clinica Ricardo Palma, Lima, Peru. DermNet NZ Editor-in-Chief: Adjunct A/Prof Amanda Oakley. Copy edited by Gus Mitchell. September 2018.


Juvenile xanthogranuloma usually presents in younger individuals as smooth pink or yellow nodules.

Histology of juvenile xanthogranuloma

In juvenile xanthogranuloma, the lesion begins as a dense lymphohistiocytic proliferation of dermis with rare giant cells. As it evolves, the dermal tumour contains a population of foamy histiocytes and giant cells (figures 1–3). Touton giant cells are quite characteristic of juvenile xanthogranuloma (best seen in figure 3). These cells contain a ring of nuclei surrounding a central homogeneous cytoplasm, while foamy cytoplasm surrounds the nuclei. Later the cells become more spindled and fibrohistiocytic-like.

Juvenile xanthogranuloma pathology

Special studies for juvenile xanthogranuloma

Immunohistochemistry can be used. Typically the tumour is positive with CD68, alpha-1-antichymotrypsin, lysozyme, vimentin, and Factor XIIIa. Useful negative stains include S100 and CD1a.

Differential diagnosis for juvenile xanthogranuloma

  • Dermatofibroma typically lacks Touton giant cells and has a dense collagenous stroma, storiform growth pattern, and often an overlying pseudoepitheliomatous hyperplasia.
  • Xanthomas show more uniform foamy histiocytes and more frequently occur in multiplicity. the polymorphous infiltrate typically seen in juvenile xanthogranuloma is usually absent.
  • Langerhans cell histiocytosis typically shows staining with S100+ and CD1a+.
  • Reticulohistiocytoma is composed of histiocytes with eosinophilic or ground glass cytoplasm.

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