α-D-mannosidase activity in histiocytosis X

A histochemical study of enzymatic activities was undertaken in five cases of histiocytosis X (two localized bone forms, two generalized forms, and one involving mainly the skin), each of which revealed characteristic structural features at the optical and ultrastructural levels. A confirmation was made of the original assumption of high acid α-D-mannosidase activity, i.e. activity described in human Langerhans intraepidermal cells (Elleder, 1975). In the control group of tumors, with the exception of urticaria pigmentosa, enzyme activity was either at trace level or altogether absent. Acid α-D-mannosidase activity therefore appears to be the first biochemical feature common to both histiocytosis X and the Langerhans cells. The significance of the finding for the present theory of the histogenesis of the above tumors is discussed.