JUVENILE XANTHOGRANULOMA - A CONGENITAL G IANT FORM

Introduction. 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. Th e nodules vary in size from a few millimetres to 1 to 2 centimetres. W e observed an exceptional case presenting as a congenital giant form. Case report. A new-born girl had a bright red lesion of the right ingu inal region measuring 4x2.5 cm. Inguinal node enlargement was found ho molaterally. Histological examination of a biopsy specimen gave the di agnosis of JGX. There was no extension. The lesion and node involvemen t regressed spontaneously leaving a xanthomization of the skin. Discus sion. Clinical forms of JGX are rarely described. The large size of th ese lesions does not apparently affect the clinical course nor associa ted visceral involvement which remains exceptional. This type of lesio n increases in size more rapidly and raises the problem of differentia l diagnosis requiring biopsy to eliminate other neonatal rumours with a less favourable prognosis. Spontaneous regression fo JGX is the rule allowing simple regular surveillance.