PMID: 3745934Jan 1, 1986Paper

Hemidesmosome heterogeneity in junctional epidermolysis bullosa revealed by morphometric analysis

The Journal of Investigative Dermatology
M J Tidman, R A Eady

Abstract

In order to examine the claim for a numerical and structural abnormality of the hemidesmosomes in junctional epidermolysis bullosa (JEB), a morphometric analysis of unseparated dermal-epidermal junction was undertaken in 11 subjects with JEB. Of these, 5 died in infancy with "lethal" disease, 3 were children still alive at 1-6 years with "indeterminate" disease, and 3 were females aged 20-60 years with variable phenotypic expression of "nonlethal" JEB. All the lethal cases had reduced numbers of hemidesmosomes which were small and lacked normal subbasal dense plates, with the exception of 1 patient whose hemidesmosomes were structurally and numerically normal. The principal hemidesmosome abnormality in the 3 cases with indeterminate JEB was the absence of normal subbasal dense plates. In 2 of the 3 cases of nonlethal JEB, the hemidesmosomes appeared normal, whereas in the third patient they showed a similar abnormality to that present in the majority of the lethal group. These results demonstrate that JEB is an ultrastructurally heterogeneous condition, and suggest that, even though the hemidesmosome abnormalities may be of diagnostic value, they do not correlate sufficiently well with the clinical outcome to be useful as a pro...Continue Reading

Citations

Oct 1, 1992·The Annals of Otology, Rhinology, and Laryngology·S BersonD M Carter
May 1, 1987·Clinical and Experimental Dermatology·R A Eady
Aug 1, 1987·Pediatric Dermatology·H Davies, D J Atherton
Sep 1, 1997·Mammalian Genome : Official Journal of the International Mammalian Genome Society·J E KusterP J Swiatek
Jan 9, 2010·Histopathology·Nikoletta Nagy, John A McGrath
Dec 10, 2014·Cell and Tissue Research·Gernot WalkoGerhard Wiche
Aug 17, 2020·Biomolecules·Svenja Kleiser, Alexander Nyström
Sep 1, 1987·The British Journal of Dermatology·A H HeagertyJ P Ortonne
Sep 21, 2004·The British Journal of Dermatology·A M G PasmooijM F Jonkman
May 16, 2019·The British Journal of Dermatology·C HasG Zambruno
Oct 23, 1997·Pediatric Dermatology·A N Lin
Feb 1, 1992·Clinical Pediatrics·S R Schaffer
Feb 1, 1996·Archives of Dermatological Research·J R McMillan, R A Eady
Jan 15, 1994·European Journal of Biochemistry·J VaillyJ P Ortonne
Nov 24, 2005·The Journal of Clinical Pediatric Dentistry·Filiz Namdar PekinerEmine C Sezen
Aug 25, 2004·Journal of the European Academy of Dermatology and Venereology : JEADV·R MallipeddiR A J Eady
May 29, 2015·Cell and Tissue Research·Gernot WalkoGerhard Wiche
Apr 1, 1993·International Journal of Dermatology·B D Zelickson, A S Zelickson
Jan 1, 1994·Archives of Dermatological Research·R A Eady, M G Dunnill
Jun 24, 1999·Journal of Dermatological Science·M F Jonkman
Oct 1, 1992·The Journal of Investigative Dermatology·M F JonkmanA Sonnenberg
Nov 1, 1993·The Journal of Investigative Dermatology·P VerrandoJ P Ortonne
Jan 1, 1989·The Journal of Investigative Dermatology·S MuellerJ R Stanley

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