Juvenile xanthogranuloma as a sequel to Langerhans cell histiocytosis: a report of three cases

We report three children who had multisystem Langerhans cell histiocytosis (LCH) with cutaneous involvement and subsequently developed juvenile xantho granuloma (JXG). JXG appeared 3 - 6 years after the initial manifestation o f - LCH. JXG lesions, which presented as yellowish papules. revealed typica l Touton giant cells and were factor XIIIa positive but S100 and CD1a negat ive. Non-LCH histiocyte disorders, such as JXG, are known to occur as a rea ction to a variety of external stimuli such as infection and trauma. It is therefore conceivable that the inflammatory reaction associated with LCH ma y have precipitated the development of JXG in our patients. Alternatively, one could speculate that this association might be due to a common histogen etic precursor of the cell types involved.