Giant congenital juvenile xanthogranuloma.

A 1-month-old otherwise healthy girl was referred to our clinic for evaluation of a congenital asymptomatic lesion on the face. Upon physical exam, a firm, sharply marginated, infiltrative, yellowish plaque measuring 6×4 cms with peau d'orange surface was evident on the right mandibular region (figure 1). A 4 mm punch biopsy was performed and histological exam revealed a diffuse infiltrate of non-foamy mononuclear histiocytes with scattered eosinophils within the papillary dermis, and a scarce amount of Touton cells. Immunohistochemistry was positive for the histiocytic marker, CD68, and negative for CD1a and S-100 protein, indicating an origin of non-Langerhans cells. On this basis, we confirmed the clinical suspicion of giant congenital juvenile xanthogranuloma (JXG)