DOI: 10.36347/sasjm.2023.v09i05.016

Pages: 461-463

Juvenile xanthogranuloma (JXG) is a proliferative disease included in the group of non-Langerhansian histiocytoses (NLH) that mainly affects infants and children, and is congenital in 40-70% of cases. This condition is usually a single or few lesions but the profuse form is very rare. We report a case of giant, profuse xanthogranuloma in a 14-month-old infant. A 5-month-old infant was brought to the dermatology department with a rash that had been flaring for more than a month. The infant had no previous history of the disease and the lesions were asymptomatic. Clinical examination revealed firm nodulopapular lesions of normal skin colour, varying in size from 5 mm to 1 cm, scattered over the body. The lesions were most prominent on the neck and trunk. Biopsy of one nodule was concluded to be a juvenile xanthogranuloma. We reassured the parents about the disease and abstained from treatment. After 9 months of evolution, the child was readmitted to dermatology for aggravation of the rash. On examination, turgid nodules of 1-2cm in diameter were observed, profuse on the body. Elsewhere the clinical and biological examination was unremarkable. We report a case of giant diffuse xanthogranuloma in a 14-month-old infant, evident by histological examination. Our observation is peculiar because of its mode of extension and the giant nature of the lesions. It remains to be seen whether the evolution of this disease passes through this turgid stage. Further work is needed to support the pathogenesis of the lesions.