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Hemosiderotic Juvenile Xanthogranuloma.

Angel Fernandez-Flores1, Ina Nicklaus, Fiona Browne

  • 1*Department of Cellular Pathology, Hospital El Bierzo, Cellular Pathology, Ponferrada, Spain; †Biomedical Investigation Institute of A Coruña, CellCOM-ST Group, A Coruña, Spain; ‡Department of Cellular Pathology, Hospital de la Reina, Cellular Pathology, Ponferrada, Spain; §Department of Histopathology, Children's Birmingham Hospital, Birmingham, United Kingdom; ¶Department of Histopathology, Birmingham Children's Hospital, Birmingham, United Kingdom; ‖Department of Histopathology, University Children's Hospital, Dublin, Ireland; and **Department of Histopathology, Hospital Niño Jesus, Madrid, Spain.

The American Journal of Dermatopathology
|April 12, 2017
PubMed
Summary

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A rare hemosiderotic variant of juvenile xanthogranuloma, a non-Langerhans cell skin condition, was identified in a pediatric patient. This unique presentation involved iron deposits within macrophages, expanding the dermis.

Area of Science:

  • Dermatopathology
  • Pediatric Pathology
  • Histiocytic Disorders

Background:

  • Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis primarily affecting the skin.
  • Distinguishing JXG from other skin lesions like dermatofibroma and Langerhans cell histiocytosis is crucial.
  • JXG can rarely occur in extracutaneous sites or be linked to systemic diseases.

Observation:

  • A 12-year-old girl presented with a slowly growing, yellowish, polypoid lesion on her scalp with occasional bleeding.
  • The lesion exhibited histopathological features of a cellular infiltrate in the dermis with a Grenz zone.
  • Epidermal changes were unremarkable.

Findings:

  • The JXG variant contained xanthomatous macrophages and Touton giant cells.

Related Experiment Videos

  • Macrophages showed abundant cytoplasmic iron deposits, indicating a hemosiderotic nature.
  • Immunohistochemistry confirmed macrophage markers (CD68, CD163) and negative markers for Langerhans cells (S100, CD1a, Langerin).

    • Implications:

    • This case describes a previously unreported hemosiderotic variant of juvenile xanthogranuloma.
    • Highlights the importance of histopathology and immunohistochemistry in diagnosing rare JXG presentations.
    • Contributes to the understanding of the diverse clinical and pathological spectrum of juvenile xanthogranuloma.