Ultrastructure of dyskeratosis in morbus Darier. 1977

A Sato, and I Anton-Lamprecht, and U W Schnyder

Biospy specimens from three patients with morbus Darier were studied by electron microscopy: Grains appeared in groups in the midst of dyskeratotic lesions and were recognized as being the result of more advanced acantholysis and precocious keratinization. Corps ronds were formed individually in the regions lateral to that of grains, where hypergranulosis was prominent in contrast to a mild dyskeratosis. Therefore, the formation of grains and corps ronds seem to be independent of each other. While in the literature acantholysis has mainly been ascribed to the separat;on of desmosones into two segments, another initial process seems to be the primary disappearance of the attachment plaque along with the connecting tonofilaments. From the existence of the other contact complexes, it seems likely that a restoration of desmosomes between acantholytic cells should take place. In early stages of dyskeratotic cells, keratinosomes were increased in number and some of them persisted inside the plasma membrane without a release into the intercellular spaces, and thus they were present in grains and corps ronds. The dyskeratosis in morbus Darier is characterized by a premature, incomplete and individual keratinization as evidenced by the existence of keratohyalin granules and keratinosomes as early as at the level of the 3rd epidermal layer and the absence of a keratin pattern in the dyskeratotic horny cells.

UI MeSH Term Description Entries
D007644 Darier Disease An autosomal dominantly inherited skin disorder characterized by warty malodorous papules that coalesce into plaques. It is caused by mutations in the ATP2A2 gene encoding SERCA2 protein, one of the SARCOPLASMIC RETICULUM CALCIUM-TRANSPORTING ATPASES. The condition is similar, clinically and histologically, to BENIGN FAMILIAL PEMPHIGUS, another autosomal dominant skin disorder. Both diseases have defective calcium pumps (CALCIUM-TRANSPORTING ATPASES) and unstable desmosomal adhesion junctions (DESMOSOMES) between KERATINOCYTES. Acrokeratosis Verruciformis of Hopf,Darier-White Disease,Keratosis Follicularis,Acantholytic Dyskeratotic Epidermal Nevi,Acantholytic Dyskeratotic Epidermal Nevus,Acrokeratosis Verruciformis,Darier's Disease,Hopf Disease,Darier White Disease,Darier-White Diseases,Dariers Disease,Disease, Darier,Disease, Darier's,Disease, Darier-White,Disease, Hopf,Diseases, Darier-White,Diseases, Hopf,Hopf Acrokeratosis Verruciformis,Hopf Diseases,Verruciformis, Acrokeratosis
D008854 Microscopy, Electron Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen. Electron Microscopy
D002462 Cell Membrane The lipid- and protein-containing, selectively permeable membrane that surrounds the cytoplasm in prokaryotic and eukaryotic cells. Plasma Membrane,Cytoplasmic Membrane,Cell Membranes,Cytoplasmic Membranes,Membrane, Cell,Membrane, Cytoplasmic,Membrane, Plasma,Membranes, Cell,Membranes, Cytoplasmic,Membranes, Plasma,Plasma Membranes
D002467 Cell Nucleus Within a eukaryotic cell, a membrane-limited body which contains chromosomes and one or more nucleoli (CELL NUCLEOLUS). The nuclear membrane consists of a double unit-type membrane which is perforated by a number of pores; the outermost membrane is continuous with the ENDOPLASMIC RETICULUM. A cell may contain more than one nucleus. (From Singleton & Sainsbury, Dictionary of Microbiology and Molecular Biology, 2d ed) Cell Nuclei,Nuclei, Cell,Nucleus, Cell
D003593 Cytoplasm The part of a cell that contains the CYTOSOL and small structures excluding the CELL NUCLEUS; MITOCHONDRIA; and large VACUOLES. (Glick, Glossary of Biochemistry and Molecular Biology, 1990) Protoplasm,Cytoplasms,Protoplasms
D003599 Cytoskeleton The network of filaments, tubules, and interconnecting filamentous bridges which give shape, structure, and organization to the cytoplasm. Cytoplasmic Filaments,Cytoskeletal Filaments,Microtrabecular Lattice,Cytoplasmic Filament,Cytoskeletal Filament,Cytoskeletons,Filament, Cytoplasmic,Filament, Cytoskeletal,Filaments, Cytoplasmic,Filaments, Cytoskeletal,Lattice, Microtrabecular,Lattices, Microtrabecular,Microtrabecular Lattices
D003896 Desmosomes A type of junction that attaches one cell to its neighbor. One of a number of differentiated regions which occur, for example, where the cytoplasmic membranes of adjacent epithelial cells are closely apposed. It consists of a circular region of each membrane together with associated intracellular microfilaments and an intercellular material which may include, for example, mucopolysaccharides. (From Glick, Glossary of Biochemistry and Molecular Biology, 1990; Singleton & Sainsbury, Dictionary of Microbiology and Molecular Biology, 2d ed) Desmosome
D004817 Epidermis The external, nonvascular layer of the skin. It is made up, from within outward, of five layers of EPITHELIUM: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis).
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000051 Acantholysis Separation of the prickle cells of the stratum spinosum of the epidermis, resulting in atrophy of the prickle cell layer. It is seen in diseases such as pemphigus vulgaris (see PEMPHIGUS) and DARIER DISEASE. Acantholyses

Related Publications

A Sato, and I Anton-Lamprecht, and U W Schnyder
March 1975, Vestnik dermatologii i venerologii,
A Sato, and I Anton-Lamprecht, and U W Schnyder
September 1966, Zeitschrift fur Haut- und Geschlechtskrankheiten,
A Sato, and I Anton-Lamprecht, and U W Schnyder
December 1970, Dermatologische Monatschrift,
A Sato, and I Anton-Lamprecht, and U W Schnyder
January 1952, Dermatologica,
A Sato, and I Anton-Lamprecht, and U W Schnyder
January 2018, Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG,
A Sato, and I Anton-Lamprecht, and U W Schnyder
July 1974, Zeitschrift fur Hautkrankheiten,
A Sato, and I Anton-Lamprecht, and U W Schnyder
April 1992, Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete,
A Sato, and I Anton-Lamprecht, and U W Schnyder
November 2018, Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG,
A Sato, and I Anton-Lamprecht, and U W Schnyder
April 1948, Nordisk medicin,
A Sato, and I Anton-Lamprecht, and U W Schnyder
November 2000, Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete,
Copied contents to your clipboard!