JUVENILE MULTIPLE XANTHOGRANULOMA IN A PATIENT WITH LANGERHANS CELL HISTIOCYTOSIS

We present the case of a 10-week-old girl who had erythematous papules with a yellowish hue from birth with diagnosis of Langerhans cell histiocytosis, that was accompanied by a lytic lesion in the skull and hepatic involvement. After several months of treatment with prednisone and vinblastine with skin and systemic improvement, several rounded erythematous papules with a yellowish hue appeared in the in right cheek. The biopsy showed a histiocytic infiltrate with positivity for CD68 and negative staining for S100 and CD1a, with a final diagnosis of juvenile xanthogranuloma. This association has been previously described in the literature in few cases. Although several hypotheses have been suggested, the causal relationship between both entities has still not been demonstrated.