II. Epidemiology

  1. Onset in infants

III. Pathophysiology

  1. Benign histiocytic tumor of childhood (non-Langerhans Cell)

IV. Signs

  1. Asymptomatic lesions
  2. Characteristics
    1. Yellow Papules and Nodules (some Nodules may be deep within the soft tissue)
    2. Typically appear as single lesions, but multiple lesions may occur
  3. Distribution
    1. Most common on the head and neck
    2. Also involve the upper torso and extremities
  4. Dermoscopy: "Setting Sun"
    1. Yellow-Orange background
    2. Foreground with yellow globules, white streaks and irregular vasculature

V. Differential Diagnosis

VI. Labs: Histopathology

  1. Lipid-laden, foamy histiocytes
  2. Scattered Eosinophils and Lymphocytes
  3. Touton-type giant cells
    1. Multinucleated cells with fused epitheloid Macrophages

VII. Course

  1. Lesions have onset in infancy and clear by adolescence

VIII. Management

  1. Solitary, Single Asymptomatic Xanthogranuloma
    1. Perform full body skin exam (evaluate for other associated lesions, findings)
    2. Observe for resolution
  2. Multiple Xanthogranulomas
    1. Abdominal Ultrasound
      1. Evaluate for hepatic xanthogranulomas (may present with Hepatomegaly, Jaundice)
    2. Ophthalmology Exam
      1. Evaluate for Eye involvement

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Ontology: Juvenile Xanthogranuloma (C0043324)

Definition (NCI) A benign histiocytic tumor that occurs during childhood; it is distinct from Langerhans cell histiocytosis. It is characterized by the presence of lipid-laden, foamy histiocytes and Touton-type giant cells in the dermis. The lesions usually develop during infancy. They consist of cutaneous papules and nodules (most often in the head and neck). It is sometimes associated with deep soft tissues nodules.
Definition (MSH) Benign disorder of infants and children caused by proliferation of HISTIOCYTES, macrophages found in tissues. These histiocytes, usually lipid-laden non-Langerhans cells, form multiple yellow-red nodules most often in the skin, the eye, and sometimes in the viscera. Patients appear to have normal lipid metabolism and are classified as a normolipemic non-Langerhans cell histiocytosis.
Concepts Neoplastic Process (T191)
MSH D014972
SnomedCT 400031009, 71709009, 189098009, 400204000
English Juvenile Xanthogranuloma, Juvenile Xanthogranulomas, Juvenile Xanthomas, Nevoxanthoendothelioma, Nevoxanthoendotheliomas, Xanthogranuloma, Juvenile, Xanthogranulomas, Juvenile, Xanthoma, Juvenile, Xanthomas, Juvenile, Juvenile Xanthoma, Multiple eruptive xanthoma, JXG - Juvenile xanthogranuloma, JXG, Xanthogranuloma, Juvenile [Disease/Finding], juvenile xanthogranuloma, juvenile xanthogranulomas, juvenile xanthoma, nevoxanthoendothelioma, xanthogranuloma juvenile, Naevoxanthoendothelioma (disorder), Juvenile xanthoma, Juvenile xanthogranuloma, Xanthoma multiplex, Congenital xanthoma tuberosum, Juvenile giant cell granuloma, Multiple eruptive xanthoma in infancy, Naevoxanthoendothelioma, Xanthoma naeviforme, Juvenile xanthogranuloma (disorder), Multiple eruptive juvenile xanthogranuloma (disorder), Multiple eruptive juvenile xanthogranuloma, Xanthoma neviforme
German Juveniles Riesenzellgranulom, Juveniles Xanthom, Xanthelasma naeviforme, Naevoxanthoendotheliom, Xanthom, juveniles, Naevoxanthom, Juveniles Xanthogranulom, Xanthoma juvenile, Xanthogranulom, juveniles
Swedish Xantogranulom, juvenilt
Czech xantom juvenilní, juvenilní xantogranulom, xantogranulom juvenilní
Finnish Juveniili ksantogranulooma
Russian KSANTOMA IUVENIL'NAIA, NEVOKSANTOENDOTELIOMA, KSANTOGRANULOMA IUVENIL'NAIA, КСАНТОГРАНУЛОМА ЮВЕНИЛЬНАЯ, КСАНТОМА ЮВЕНИЛЬНАЯ, НЕВОКСАНТОЭНДОТЕЛИОМА
Italian Nevoxantoendotelioma, Xantoma giovanile, Xantogranuloma giovanile
Polish Kępki żółte młodocianych, Żółtak młodocianych
Japanese 黄色肉芽腫-若年性, 黄色腫-若年性, 母斑性黄色内皮腫, 若年性黄色肉芽腫, 若年性黄色腫
Spanish xantoma juvenil, nevoxantoendotelioma, xantogranuloma juvenil (trastorno), xantogranuloma juvenil eruptivo múltiple (trastorno), xantogranuloma juvenil eruptivo múltiple, xantogranuloma juvenil, Nevoxantoendotelioma, Xantogranuloma Juvenil, Xantoma Juvenil
Norwegian Juvenilt xantom, Juvenilt xantogranulom
French Naevo-xantho-endothéliome, Xanthogranulome juvénile, Xanthome juvénile
Dutch Naevoxantho-endothelioom, Xanthogranuloma juvenilis, Xanthoma juvenilis
Portuguese Nevoxantoendotelioma, Xantogranuloma Juvenil, Xantoma Juvenil