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A Patient-Derived Xenograft Model for Venous Malformation
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Published on: June 15, 2020

Segmental juvenile xanthogranuloma.

Su Yuen Ng1

  • 1Institut Pediatrik, Hospital Kuala Lumpur, Kuala Lumpur, Malaysia.

Pediatric Dermatology
|July 30, 2013
PubMed
Summary
This summary is machine-generated.

This study reports an unusual case of juvenile xanthogranuloma in an infant. The condition presented as skin nodules with a rare segmental distribution, confirmed by histopathology.

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Published on: June 15, 2020

Area of Science:

  • Dermatology
  • Pediatric Pathology
  • Histopathology

Background:

  • Juvenile xanthogranuloma (JXG) is a common benign xanthogranulomatous condition typically presenting in infants and young children.
  • JXG lesions are usually solitary or multiple, non-Langerhans cell histiocytoses.
  • Atypical presentations of JXG, including segmental distribution, are rare.

Observation:

  • A 5-month-old boy presented with erythematous nodules on the left side of his trunk since birth.
  • The nodules exhibited a distinct segmental arrangement, following Blaschko's lines.
  • Clinical presentation suggested a possible inflammatory or neoplastic process.

Findings:

  • Histopathologic examination revealed dense dermal and subcutaneous infiltration by sheets of foamy histiocytes.
  • Multinucleated giant cells and lymphocytes were admixed within the histiocyte infiltrate.
  • The findings were consistent with juvenile xanthogranuloma, characterized by its unusual segmental distribution.

Implications:

  • This case highlights the importance of considering JXG in the differential diagnosis of segmental skin lesions in infants.
  • The segmental pattern may represent a unique manifestation of JXG, potentially linked to somatic mosaicism.
  • Further research into the genetic and developmental basis of segmental JXG is warranted to understand its pathogenesis.