2000 Volume 5 Issue 2 Pages 87-94
Pemphigus is an autoimmune disease in which autoantibodies play a pathogenic role, causing loss of epithelial cell adhesion with resulting blister formation. In the present study, the distribution patterns of desmocollin 3 (Dsc 3), desmocollin 1 (Dsc 1) and desmoglein 1 and 2 (Dsg 1,2) in pemphigus vulgaris (PV) and pemphigus erythematosus (PE) were investigated by immunohistochemistry. Dsc 3 was distributed at the cell membrane of the basal and suprabasal layers of the normal oral mucosa and skin epithelium. Dsc 1 was distributed only at the cell membrane of the upper spinous layers of normal skin, and was not visible in normal oral mucosa epithelium. Dsg 1,2 was distributed at the cell membrane of the suprabasal layers of normal oral mucosa and skin epithelium. The distribution of Dsc 3 and Dsg 1,2 was obviously sparser in the oral PV and cutaneous PV than in normal control epithelium. Internalization of Dsc 3, Dsc 1 and Dsg 1,2 occurred specifically in PV and PE, giving staining patterns resembling cytoplasmic patchy granules, rings and dense clumps. Internalization of Dsc 3 was more frequent in cutaneous PV (60%) and PE (60%) than in oral PV (30%). These findings suggest that internalization of Dsc 3 is closely associated with disease duration (P<0.01) and disease severity (P<0.05) in PV, and that not only desmogleins but also desmocollins may play an etiological role.
