Subcutaneous juvenile xanthogranuloma.

A 1-month-old female infant was seen in consultation because of a congenital subcutaneous nodule, 2 x 3 cm in diameter, in the right retroauricular area. The nodule was surgically excised. Histologically, a large subcutaneous nodular mass was well delimited but not encapsulated. It consisted of a monomorphous sheet of foamy histiocytic cells with scattered eosinophils. Multinucleated giant cells were not found. The pure subcutaneous type of juvenile xanthogranuloma has been reported very rarely in the literature. The usual histological monomorphism of these cases, with only occasional eosinophils and lymphocytes and rare Touton giant cells, adds another diagnostic difficulty to their unusual clinical presentation.