Self-regressing S100-negative CD1a-positive cutaneous histiocytosis

In the skin, the antigen-presenting cells are mainly represented by Langerhans cells, indeterminate cells, and interstitial dendritic cells, which show distinctive immunophenotype and/or ultrastructure. We report a case of a cutaneous-limited self-regressing histiocytosis with a peculiar immunohistochemical profile (CD1a-positive and S100 protein-negative) that is not observed in any of the known cutaneous antigen-presenting cell or nowadays recognized neoplasm. This lesion is probably related to indeterminate dendritic cell tumors, but very few cases with such immunoprofile have been reported up-to-date, and their exact nosologic position and outcome remain to be clarified.