Solitary giant xanthogranuloma and benign cephalic histiocytosis — variants of juvenile xanthogranuloma

Abstract

Summary Sequential biopsies taken from a patient with a solitary giant xanthogranuloma, an exaggerated macronodular (>5 cm in diameter) variant of juvenile xanthogranuloma, and from a patient with benign cephalic histiocytosis, revealed a characteristic time sequence of histopathological findings. Early stages of the diseases showed a monomorphous infiltrate of mononuclear vacuolated histiocytes positive for KiM1p. HAM56 and factor XIIIa and were characterized by clusters of comma‐shaped bodies. This was followed by a polymorphous mixture of various mononuclear and mullinucleate histiocytes additionally labelling with KP1 (CD68) and, in occasional cells, for the adherence of peanut agglutinin. A variety of ultrastructural changes were found, including dense and regularly laminated bodies or lipid droplets. Our findings indicate that both entities are variants of a xanthogranulomatous reaction.