[Juvenile xanthogranuloma: a congenital giant form]

Juvenile giant xanthogranuloma (JGX) is usually a benign skin disease which regresses spontaneously in the new-born or infant. Characteristic firm yellow-orange papulonodules lead to diagnosis. The nodules vary in size from a few millimetres to 1 to 2 centimetres. We observed an exceptional case presenting as a congenital giant form.

A new-born girl had a bright red lesion of the right inguinal region measuring 4 x 2.5 cm. Inguinal node enlargement was found homolaterally. Histological examination of a biopsy specimen gave the diagnosis of JGX. There was no extension. The lesion and node involvement regressed spontaneously leaving a xanthomization of the skin.

Clinical forms of JGX are rarely described. The large size of these lesions does not apparently affect the clinical course nor associated visceral involvement which remains exceptional. This type of lesion increases in size more rapidly and raises the problem of differential diagnosis requiring biopsy to eliminate other neonatal tumours with a less favourable prognosis. Spontaneous regression of JGX is the rule allowing simple regular surveillance.