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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 pathology — codes and concepts


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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