CD68-negative nonlipidized juvenile xanthogranuloma

Abstract Juvenile xanthogranuloma (JXG) belongs to the group of non-Langerhans cell histiocytosis. JXG is typically factor XIIIa- and CD68-positive. Nonlipidized JXG (NJXG) is a rare, mononuclear variant of JXG that shows few or absence of foam cells or Touton giant cells in the histiocytic infiltrate. Herein, we describe an unusual case of NJXG in a 7-year-old boy presenting with a 1-month history of a flesh-colored nodule on the forehead. The lesion was factor XIIIa-positive and CD68-negative but CD163-positive. Our case illustrates that CD163, a more specific marker of macrophage and monocyte differentiation, is a valuable marker for the diagnosis of NJXG.