Intermediate cell histiocytosis: a case report of a rare disorder

Abstract

Histiocytoses are a heterogeneous group of disorders including: X, non-X, as well as the rare intermediate cell histiocytosis. This classification is based on clinicopathological as well as ultrastructural aspects. Intermediate cells belong to the dendritic cell group involved in antigen presentation to lymphocytes. These cells characteristically express S-100 and CD1a and lack Birbeck granules. We report a case of an 80 year old male with intermediate cell histiocytosis. This patient presented with multiple pruritic firm papules and nodules on the bilateral anterior lower extremities. An extensive systemic work-up revealed only a single lytic lesion in the skull. He received high potency topical steroids with good resolution of his pruritus, but with no effect on the cutaneous nodules. He has remained without systemic involvement after one year and the lytic lesion remained unchanged.