DESMOSOMAL DISSOLUTION IN GROVERS DISEASE, HAILEY-HAILEYS DISEASE ANDDARIERS-DISEASE

Proteins involved in the formation of desmosomes and simpler adherens junctions were studied in three types of non-immune acantholytic disea ses; specifically; four cases of Grover's disease (GD), one case of Ha iley-Hailey's disease (HHD) and one case of Darier's disease (DD), and these were compared to two cases of immune-mediated acantholytic dise ase pemphigus vulgaris (PV). The proteins studied included: 1. The int racellular desmosomal proteins, desmoplakin I and II and plakoglobin; 2. The intercellular desmosomal proteins, desmoglein and CD44; and 3. vinculin, which is a major intracellular protein of the simpler aheren s junctions. In GD, I-Ii-ID and DD, immunostaining showed a loss of de smoplakin I and II and plakoglobin from the desmosomes, and a diffuse staining in the cytoplasm. In contrast, in pemphigus vulgaris, these p roteins seemed intact and were localized to dot-like spots on the cell surface. Also, desmoglein, and CD44 were slightly affected in GD, and moderately affected in HHD and DD. Absence of desmosomal attachment p laques, the lack of labeling with desmoglein in the affected desmosome s and a diffusion of the labels into cytoplasm were demonstrated with electron microscopy using an immunogold technique, In PV desmoglein II I is one of the target antigens for the autoantibodies in this disease and was only partially presented in a small number of lesional cells, while CD44 was mostly preserved, Vinculin was intact in GD, HHD and D D, but was lost in PV. This study, our previous work, and that of othe rs, suggest that: 1. In GD, HHD and DD, the proteins of the desmosomal attachment plaque are primarily affected; 2. In PV, the intercellular glycoproteins are primarily involved; and 3. Simple adherens junction s are intact in GD, HHD and DD, but are damaged in PV.