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Subcutaneous juvenile xanthogranuloma

E Sánchez Yus1, L Requena, C Villegas

  • 1Department of Dermatology, Hospital Universitario San Carlos, Facultad de Medicina, Universidad Complutense, Madrid, Spain.

Journal of Cutaneous Pathology
|October 1, 1995
PubMed
Summary
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A rare congenital subcutaneous nodule in an infant was diagnosed as juvenile xanthogranuloma. Histology revealed foamy histiocytic cells, highlighting diagnostic challenges of this rare presentation.

Area of Science:

  • Dermatology
  • Pediatric Pathology

Background:

  • Juvenile xanthogranuloma (JXG) is a rare, benign histiocytic proliferation typically affecting infants and young children.
  • While commonly presenting as cutaneous or subcutaneous nodules, the pure subcutaneous form is exceptionally uncommon.

Observation:

  • A 1-month-old female infant presented with a congenital 2 x 3 cm subcutaneous nodule in the right retroauricular area.
  • The nodule was surgically excised for diagnostic and therapeutic purposes.

Findings:

  • Histopathological examination revealed a well-delimited, non-encapsulated subcutaneous mass.
  • The lesion was composed of a monomorphous sheet of foamy histiocytic cells with scattered eosinophils.
  • Notably, multinucleated giant cells, including Touton giant cells, were absent, contributing to histological monomorphism.

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Implications:

  • This case underscores the rarity of the pure subcutaneous variant of juvenile xanthogranuloma.
  • The histological monomorphism, with minimal cellular atypia and absence of giant cells, presents diagnostic challenges.
  • Accurate histological diagnosis is crucial for appropriate management and to differentiate from other pediatric soft tissue tumors.