Struktur und Schicksal der Desmosomen beim Morbus Darier

Abstract

Atypical desmosomes are found between cells at the bottom of the lacunae in Darier’s disease. The spacing between the intermediate dense lines may be irregularly reduced, and the extracellular material can exhibit an uneven moth-eaten aspect; the intercellular contact layer is missing, but tonofibrils are connected to the attachment plaques. These desmosomes are interpreted as malformations. Desmosomes with a dilated intercellular gap are expected to split in two halves.

Ultrastructure of Dyskeratosis in Morbus Darier

“…The first claims that tonofilaments separate from the attachment plaques and thereafter desmosomes disappear (Charles 1961, Caulfield & Wilgram 1963, Wilgram & Weinstock 1966, Pierard & Kint 1968; the same can also occur in one segment of desmosomes alone (Caulfield & Wilgram 1963). According to the second interpretation, the desmosomes loose their interceullar contact layer and thus puU apart into two halves, after which tonofilaments become detached from the desmosomes (Mann & Haye 1970, Arai 1971, Vogel & Narasimhan 1974, Biagini et al 1975, Peck et al 1976. In our study there were fair numbers of one half of desmosomes attached to the plasma membrane of acantholytic cells (Fig.…”