Generalized histiocytosis X in the elderly: a light and electron microscope and monoclonal antibody study.
Two elderly female patients are described with generalized histiocytosis X (Letterer-Siwe disease). In each case, a definitive diagnosis was not established until ultrastructural and immunoperoxidase investigations had been performed. The histopathological findings in skin biopsies from each patient were similar. Light microscopy demonstrated a bandlike epidermotrophic cellular infiltrate which included large atypical cells (histiocytosis X cells). Electron microscopy showed that these cells contained Birbeck granules. Monoclonal antibody studies demonstrated the presence of T6, T4 and HLA-DR surface antigens. Lysozyme and alpha-I-antitrypsin were absent from the cells. The associated cellular infiltrate included T4 and T8 positive lymphocytes. It is possible that more cases of generalized histiocytosis X in adults will be identified with the increasing use of specialized histopathological techniques and that the disease is more common than currently believed.
D000368:Aged; D000911:Antibodies, Monoclonal; D001706:Biopsy; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000473:pathology*; D006801:Humans; D007124:Immunoenzyme Techniques; D008854:Microscopy, Electron; D012867:Skin / Q000473:pathology / Q000648:ultrastructure
D000911:Antibodies, Monoclonal