Document Detail

Ultrastructural studies in epidermolysis bullosa heriditaria. I. Dominant dystrophic type of Pasini.
MedLine Citation:
PMID:  1155959     Owner:  NLM     Status:  MEDLINE    
Ultrastructural examination was performed in 8 biopsies from 4 patients with the Pasini type of epidermolysis bullosa dystrophica dominans. The biopsies were taken from: 1. clinically normal skin from nonpredilection areas, 2. intact skin from predilection areas, 3. involved skin and 4. experimentally frictioned skin. The main ultrastructural alterations detected are as follows: hypoplasia of anchoring fibrils, split or blister formation between basal lamina and dermis, hernia-like protrusion of basal cells, sub- and intraepidermal deposition of fibrillar bodies, and duplications of basal lamina. Among them, the constantly observed finding in all of the four biopsy groups in the structural defect of anchoring fibrils, namely, that the anchoring fibrils are rudimentary and reduced in number. Presence of the structural defect of anchoring fibrils in clinically normal skin from nonpredilection areas in patients with the Pasini type of epidermolysis bullosa dystrophica dominans indicates that this defect is not a secondary change following repeated mechanical trauma, but a primary, genetically determined event.
I Hashimoto; I Anton-Lamprecht; T Gedde-Dahl; U W Schnyder
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Archiv für dermatologische Forschung     Volume:  252     ISSN:  0003-9187     ISO Abbreviation:  Arch Dermatol Forsch     Publication Date:  1975  
Date Detail:
Created Date:  1975-10-28     Completed Date:  1975-10-28     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7512588     Medline TA:  Arch Dermatol Forsch     Country:  GERMANY, WEST    
Other Details:
Languages:  eng     Pagination:  167-78     Citation Subset:  IM    
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MeSH Terms
Connective Tissue / ultrastructure
Connective Tissue Cells
Epidermolysis Bullosa / genetics,  pathology*
Skin / ultrastructure
Stress, Mechanical

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