engleski

Juvenile xanthogranuloma in a newborn

Juvenile xanthogranuloma (JXG) presents a normolipemic non-Langerhans cells histiocytosis.(5) JXG usually presents with cutaneous lessions. Visceral involment is rare but may affect varrious organs. Deep JXG differs from the cutaneous form histologically by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. Awareness of the possibility of this atypical presentation of the JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen).(8) JXG may present with intramuscular lessions only, however, rarely JXG has been previously reported to affect the heart, but not without the typical cutaneous manifestations.(1, 9) We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. According to our knowledge this is a first case of intracavitar JXG without skin lesions.

neonate; heart; juvenile xanthogranuloma; pediatrics

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