Scanning electron microscopy of a blister roof in dystrophic epidermolysis bullosa

被引:1
作者
de Almeida, Hiram Larangeira, Jr. [1 ,2 ]
Monteiro, Luciane [3 ]
Marques e Silva, Ricardo [4 ]
Rocha, Nara Moreira [5 ]
Scheffer, Hans [6 ]
机构
[1] Univ Fed Pelotas, Pelotas, RS, Brazil
[2] Catholic Univ Pelotas UFPel, Pelotas, RS, Brazil
[3] Catholic Univ Pelotas UFPel, Postgrad Program Hlth, Pelotas, RS, Brazil
[4] Fed Univ Pelotas UFPel, Lab Electron Microscopy, Pelotas, RS, Brazil
[5] Empresa Brasileira Pesquisa Agr EMBRAPA CPA CT, Electron Microscopy Lab, Pelotas, RS, Brazil
[6] Radboud Univ Nijmegen, Med Ctr, DNA Diagnost Div, Dept Human Genet, NL-6525 ED Nijmegen, Netherlands
关键词
Basement membrane; Epidermolysis bullosa dystrophica; Microscopy; electron; scanning; Skin diseases; vesiculobullous; MUTATION;
D O I
10.1590/abd1806-4841.20131743
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
In dystrophic epidermolysis bullosa the genetic defect of anchoring fibrils leads to cleavage beneath the basement membrane, with its consequent loss. We performed scanning electron microscopy of an inverted blister roof of a case of dystrophic epidermolysis bullosa, confirmed by immunomapping and gene sequencing. With a magnification of 2000 times a net attached to the blister roof could be easily identified. This net was composed of intertwined flat fibers. With higher magnifications, different fiber sizes could be observed, some thin fibers measuring around 80 nm and thicker ones measuring between 200 and 300 nm.
引用
收藏
页码:966 / 968
页数:3
相关论文
共 9 条
[1]   The structure of the interstitial surfaces of the epithelial basement membranes of mouse oral mucosa, gingiva and tongue [J].
Abe, M ;
Osawa, T .
ARCHIVES OF ORAL BIOLOGY, 1999, 44 (07) :587-594
[2]   Mutation analysis and characterization of COL7A1 mutations in dystrophic epidermolysis bullosa [J].
Dang, Ningning ;
Murrell, Dedee F. .
EXPERIMENTAL DERMATOLOGY, 2008, 17 (07) :553-568
[3]   Scanning electron microscopy of acantholysis in pemphigus foliaceus [J].
de Almeida, Hiram Larangeira, Jr. ;
Leitao, Aline Hatzenberger ;
Rossi, Gabriela ;
Rocha, Nara Moreira ;
Marques e Silva, Ricardo .
ANAIS BRASILEIROS DE DERMATOLOGIA, 2013, 88 (03) :456-458
[4]   The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB [J].
Fine, Jo-David ;
Eady, Robin A. J. ;
Bauer, Eugene A. ;
Bauer, Johann W. ;
Bruckner-Tuderman, Leena ;
Heagerty, Adrian ;
Hintner, Helmut ;
Hovnanian, Alain ;
Jonkman, Marcel E. ;
Leigh, Irene ;
McGrath, John A. ;
Mellerio, Jemima E. ;
Murrell, Dedee E. ;
Shimizu, Hiroshi ;
Uitto, Jouni ;
Vahlquist, Anders ;
Woodley, David ;
Zambruno, Giovanna .
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY, 2008, 58 (06) :931-950
[5]   Collagens [J].
Gordon, Marion K. ;
Hahn, Rita A. .
CELL AND TISSUE RESEARCH, 2010, 339 (01) :247-257
[6]   The clinical spectrum of dystrophic epidermolysis bullosa [J].
Horn, HM ;
Tidman, MJ .
BRITISH JOURNAL OF DERMATOLOGY, 2002, 146 (02) :267-274
[7]   The G2028R glycine substitution mutation in COL7A1 leads to marked inter-familiar clinical heterogeneity in dominant dystrophic epidermolysis bullosa [J].
Nakamura, H ;
Sawamura, D ;
Goto, M ;
Sato-Matsumura, KC ;
LaDuca, J ;
Lee, JYY ;
Masunaga, T ;
Shimizu, H .
JOURNAL OF DERMATOLOGICAL SCIENCE, 2004, 34 (03) :195-200
[8]   Immunological mapping in hereditary epidermolysis bullosa [J].
Prado de Oliveira, Zilda Najjar ;
Perigo, Alexandre M. ;
Fukumori, Ligia M. I. ;
Aoki, Valeria .
ANAIS BRASILEIROS DE DERMATOLOGIA, 2010, 85 (06) :856-861
[9]   Collagen Structure and Stability [J].
Shoulders, Matthew D. ;
Raines, Ronald T. .
ANNUAL REVIEW OF BIOCHEMISTRY, 2009, 78 :929-958