Abstract
Chronic granulomatous disease (CGD) is an intrinsic defect of phagocytic cells resulting in decreased capacity to kill certain bacteria and is a consistent clinical syndrome. Defective phagocytosis is present in a milder form in female carriers who are, in general, asymptomatic. Tubular reticular structures (TRS) have been demonstrated in peripheral lymphocytes and monocytes in patients with discoid lupus erythematosus (DLE). Recently we became aware of 4 boys with CGD who had mothers with DLE. Therefore, we examined the peripheral mononuclear cells by electron microscopy from a series of males with CGD, their carrier sisters, and their mothers, both with and without DLE, to ascertain the significance of TRS in these two disease
These studies suggest that patients with CGD and DLE share an abnormality, which may be an additional abnormality to the established phagocytic defect in CGD. The CGD carrier state may predispose to DLE. Further investigation of phagocytosis in patients with DLE and electron microscopic examination of peripheral mononuclear cells in CGD should be performed.
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Goodman, J., Wara, D., Oche, H. et al. TUBULAR RETICULAR STRUCTURES IN PERIPHERAL MONONUCLEAR CELLS OF MALES WITH CHRONIC GRANULOMATOUS DISEASE, THEIR MOTHERS, AND SISTER CARRIERS. Pediatr Res 8, 413 (1974). https://doi.org/10.1203/00006450-197404000-00439
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DOI: https://doi.org/10.1203/00006450-197404000-00439