A Case of Weber-Cockayne Epidermolysis Bullosa Simplex - Ultrastructural Findings / 대한피부과학회지
Korean Journal of Dermatology
;
: 214-219, 1997.
Article
in Korean
| WPRIM
| ID: wpr-47529
ABSTRACT
Epidermolysis bullosa simplex (EBS) is a group of autosomal dominantly inherited genetic disorders characterized by blistering due to mechanical- stress-induced degeneration of basal epiderrnal cells. Recently, it was discovered that EBS is induced by keratin 5 and 14 gene mutations. Weber Cockayne (W-C) EBS is the mildest type, with blistering concentrates primarily on palar and plantar regions, and basal cell cytolysis by keratin filament perturbations is present. Herein we report a case of W-C EBS with its ultrastructural findings. Electron microscopy showed cytolysis and separation of the basal epidermal cells, mainly at the subnuclear cytoplasm. The cyto- plasm of basal cells showed edema, loosening and intact rnitochondria. Besides the cytoplasmic changes, the nucleus also showed lytic degeneration. Characteristically, dense condensation of tonofilarnent was observed, which suggests that W-C EBS is. also a disorder of keratin.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Microscopy, Electron
/
Epidermolysis Bullosa Simplex
/
Blister
/
Epidermolysis Bullosa
/
Cytoplasm
/
Edema
/
Keratin-5
Type of study:
Diagnostic study
Language:
Korean
Journal:
Korean Journal of Dermatology
Year:
1997
Type:
Article
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