Histiocytosis-X cells express C3b, C3d, and C3bi receptor (CR1, CR2, and CR3) antigens.

Abstract

Listen

Translate article

Histiocytosis-X cells in the skin and other tissues share, with Langerhans cells (LC) of the epidermis, the membrane expression of the T6 and HLA-DR antigens [1, 2, 4] and the presence, in their cytoplasm, of an ultrastructural marker, the Birbeck granule [5]. In view of these similarities, hisfiocytosis-X cells are, at present, considered to represent an authentic proliferation of LCs or of cells derived from LCs. Histiocytosis-X cells and LCs express membrane receptors for the Fc fragment of IgG and for cleavage fragments of C3, as is demonstrated by the ability of these cells in suspension to form rosettes with antibodyand C3-bearing cellular intermediates [3, 10, 131. Using monoclonal antibodies (Mcab) specific for the human C3b receptor (CR1), C3d receptor (CR2), and the e-chain of the C3bi receptor (CR3), we demonstrated, by indirect immunofluorescence (IF) and immunoperoxidase (IPo) techniques, the presence of antigenic determinants of the three types of C3 receptors on the membrane of histiocytosis-X cells in the skin. Skin biopsies were taken from a 9-month-old infant, who had originally been referred to our department with Letterer-Siwe disease [i]. Initially, the child had diffuse lymphadenopathies and a typical papular eruption predominantly localized on the trunk and scalp. He presented crusty lesions coalescing to large plaques in the lumbar region; the papules were erosive and infiltrated in the inguinal region. After 8 weeks of follow-up, a granular interstitial infiltrate involving both lungs was seen on a chest X-ray; radiographic