1996
DOI: 10.1111/1523-1747.ep12331167
|View full text |Cite
|
Sign up to set email alerts
|

Hailey-Hailey Disease Keratinocytes: Normal Assembly of Cell-Cell Junctions In Vitro

Abstract: The blisters in the inherited disorder, Hailey-Hailey disease, may be caused by defective epidermal junctional complexes. We evaluated these structural complexes in vivo and in vitro. We induced a vesicular lesion in the apparently normal skin of a patient with Hailey-Hailey disease and studied a biopsy of this lesion by transmission electron microscopy. To determine whether acantholysis was related to a defect in the number or assembly of intercellular junctions, we cultured Hailey-Hailey disease keratinocyte… Show more

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...
2
1

Citation Types

2
10
0

Year Published

1999
1999
2014
2014

Publication Types

Select...
8
1

Relationship

0
9

Authors

Journals

citations
Cited by 13 publications
(12 citation statements)
references
References 39 publications
2
10
0
Order By: Relevance
“…21 Nevertheless, HHD and normal keratinocytes did not differ in their ability to assemble desmosomes and adherens junctions. 22 No significant morphological difference between normal and HH keratinocytes was observed in culture in low (0AE1 mmol L )1 ) and high (1AE1 mmol L )1 ) Ca 2+ -containing media. In these keratinocytes the major desmosomal proteins are biochemically intact.…”
Section: Discussionmentioning
confidence: 88%
See 1 more Smart Citation
“…21 Nevertheless, HHD and normal keratinocytes did not differ in their ability to assemble desmosomes and adherens junctions. 22 No significant morphological difference between normal and HH keratinocytes was observed in culture in low (0AE1 mmol L )1 ) and high (1AE1 mmol L )1 ) Ca 2+ -containing media. In these keratinocytes the major desmosomal proteins are biochemically intact.…”
Section: Discussionmentioning
confidence: 88%
“…In DD and HHD, immunostaining showed a loss of the intracellular desmosomal proteins (desmoplakins 1 and 2 and plakoglobin) from desmosomes and a diffuse staining in the cytoplasm 21 . Nevertheless, HHD and normal keratinocytes did not differ in their ability to assemble desmosomes and adherens junctions 22 . No significant morphological difference between normal and HH keratinocytes was observed in culture in low (0·1 mmol L −1 ) and high (1·1 mmol L −1 ) Ca 2+ ‐containing media.…”
Section: Discussionmentioning
confidence: 90%
“…Also, inflammatory infiltrates, consisting of lymphocytes and neutrophils with accompanying eosinophils are present in the superficial dermis and in the epidermis when bacterial superinfection is present (10). Direct immunofluorescence test of the skin is negative (11).…”
mentioning
confidence: 99%
“…It is hypothesized that a defect in keratinocyte adhesion predisposes the skin to breakdown and blistering. This defect may be secondary to a mutation in a calcium pump impairing phosphorylation of adhesion proteins 16 . Familial benign pemphigus has a waxing and waning course, with patients often not being affected until the third or fourth decade.…”
Section: Hailey‐hailey Diseasementioning
confidence: 99%