Juvenile xanthogranuloma

Juvenile xanthogranulomas are rare, benign tumours of histiocytic cells that occur predominantly in infancy and early childhood and spontaneously regress.

This chapter is set out as follows:

• The cause is unknown
• Cells express the phenotype of the dermal dendrocyte

• Normally present before the age of one year, 20% of cases are found at birth. Up to 10% of cases present in adults
• More common in males and Caucasians
• Lesions can develop quickly and are often single to a few in number, although occasionally large numbers develop
• The number and size of lesions tend to increase over the first 18 months of life

Distribution

• Lesions on the head, neck and trunk predominate, although can arise on any site

Morphology

• Size varies from a few mm to several cm
• Colour - initially red and then become tan-orange
• Shape - smooth dome-shaped papules and nodules
• Over time surface telangiectasia can develop
• Palpation - firm 

Non-cutaneous features

• The mouth and eyes can be affected
• Although internal organs can be affected, systemic manifestations are rare

Reassurance

• Lesions tend to regress over 1-5 years
• Provide a patient information leaflet

Ocular involvement

• Eye involvement can lead to a number of complications
• Although routine screening is no longer recommended, any patient suspected to have ocular involvement should be referred promptly to an ophthalmologist