Cicatricial Pemphigoid Antigen Differs from Bullous Pemphigoid Antigen by Its Exclusive Extracellular Localization: A Study by Indirect Immunoelectronmicroscopy

Several components of the dermal – epidermal junction (DBJ) bear the name of the autoimmune bullous disease in which they are involved. The epidermolysis bullosa acquisita (EBA) antigen, a component of anchoring fibrils, and the bullous pemphigoid (BP) antigen, a component of hemidesmosomes (HD) with a molecular weight of 220-240 kD, have been well characterized. In contrast, there is little data known about the cicatricial pemphigoid (CP) antigen. No differences between CP and BP have been reported when sera of patients were studied by Western immunoblotting. Findings of a study of sera from 8 patients with CP by indirect immunoelectron microscopy (IEM) on normal human skin are reported. Saponin (0.1% 10 mn) was used as a permermeabilizing agent of cytomembranes and saponin-treated skin samples were compared to saponin-untreated skin samples. Four sera from patients with BP, one from a patient with EBA, and three from healthy donors served as controls. The CP sera produced a similar staining of DEJ on both saponin-treated and saponin-untreated skin samples: immune deposits were localized over the lamina densa and the lower part of the lamina lucida clearly separated from the cytoplasmic membrane of keratinocytes, in regularly spaced clumps. The BP sera produced an intense staining of DEJ only on saponin-treated skin samples: immune deposits were observed on the cytoplasmic attachment plaque of the HD; on saponin-untreated skin samples, BP sera produced only a faint staining of the extracellular part of HD. Finally, as expected the EBA serum appeared on the lower part of the lamina densa and anchoring fibrils, and no DAB deposits were observed with the serum of healthy donors. This data indicated that CP antigen is different than BP antigen by its exclusive extracellular localization. It may be a component of anchoring filaments