Juvenile xanthogranuloma

26 June 2013, by KUPFER-BESSAGUET I. & WOLTER M. & DE PROST Y.

1 - BACKGROUND

Juvenile xanthogranuloma is a benign self-limiting pathology, characterised by papulonodular cutaneous lesions that are yellow-orange in colour and may be single or multiple. It is mainly infants and young children that are affected, although cases have been reported in adults. Histologically, the lesions are made up of a proliferation of histiocytes without the markers for Langerhans cell histiocytosis being expressed. Lipid metabolism is normal. The prognosis in classic cutaneous forms is excellent, with lesions regressing spontaneously within a few months or years.

2 - INFORMATION FOR PATIENTS

Juvenile xanthogranuloma:

– is a benign pathology in children of unknown aetiology;

– it consists of a yellow-orange lesion that appears to be “placed” on the skin;

– in the majority of cases, extra-cutaneous sites are not affected;

– ocular involvement is possible when the patient has multiple cutaneous lesions and is under the age of two; the signs that must be taken as a warning of this are a red and painful eye;

– an association with neurofibromatosis type 1 is possible;

– as a rule, it regresses spontaneously in the first few years of life.

 

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