Solitary cutaneous dendritic cell tumor in a child: Role of dendritic cell markers for the diagnosis of skin Langerhans cell histiocytosis

We describe a child with a solitary dendritic cell (DC) tumor positive for S-100 protein, CD1a, and HLA-DR with the clinical and histopathologic features of a so-called solitary variant of congenital self-healing Hashimoto-Pritzker reticulohistiocytosis (CSHRH). CSHRH is a spontaneously regressing, benign form of Langerhans cell histiocytosis (LCH) and was thought to be a histiocytosis consisting of precursor Langerhans cells. In our study the tumor cells did not express CD68, indicating that they represent mature DCs. Because of the negative finding for Langerin, it cannot be assessed whether the tumor consists of terminally mature Langerhans cells that have lost Langerin expression upon maturation or of mature dermal DCs. This case demonstrates that the progress in DC biology necessitates reevaluation of our knowledge of LCH to better understand the different variants of the disease. Therefore the literature on CSHRH is reviewed in light of present knowledge on cutaneous DC immunology.