ESOPHAGEAL INVOLVEMENT IN PEMPHIGUS-VULGARIS - A DIRECT AND INDIRECT IMMUNOELECTRON MICROSCOPIC STUDY

Esophageal involvement during pemphigus vulgaris (PV) has rarely been reported. Moreover, this particular form of PV has only been studied by immunofluorescence. To determine the precise in vivo and in vitro ultrastructural location on esophageal cell membranes, of anti-intercellular substance (ICS) antibodies, we analyzed using direct and indirect immunoelectron microscopy (IEM), esophaqeal biopsy and serum samples from four patients with PV and esophageal involvement. Direct IEM on patients' esophageal mucosae showed that IgG deposits were strictly localized to separated desmosomal structures on the free surface of acantholytic esophageal cells. Indirect IEM of normal human esophageal mucosa labeled with sera from 3 patients demonstrated IgG deposits in the extracellar part of desmosomal regions but also along the esophageal cell plasma membrane. This study enabled the precise localization of esophageal anti-epithelial cell surface antibody deposits in patients with PV and esophageal involvement. Moreover, it suggests that the acantholytic process may begin in the interdesmosomal regions of the esophageal cell membrane.