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Classical Ehlers-Danlos syndrome: clinical, Histological and ultrastructural aspects / Síndrome de Ehlers-Danlos clássica: aspectos clínicos, histológicos e ultraestruturais
Bicca, Eduardo de Barros Coelho; Almeida, Fabiano Bonow de; Pinto, Giselle Martins; Castro, Luis Antônio Suíta de; Almeida Júnior, Hiram Larangeira de.
  • Bicca, Eduardo de Barros Coelho; Catholic University of Pelotas. Pelotas. BR
  • Almeida, Fabiano Bonow de; Catholic University of Pelotas. Pelotas. BR
  • Pinto, Giselle Martins; Santa Casa de Misericórdia Porto Alegre. Porto Alegre. BR
  • Castro, Luis Antônio Suíta de; EMBRAPA. CPA-CT Pelotas. electronic microscopy laboratory. Pelotas. BR
  • Almeida Júnior, Hiram Larangeira de; Catholic University of Pelotas. Pelotas. BR
An. bras. dermatol ; 86(4,supl.1): 164-167, jul,-ago. 2011. ilus
Article Dans Anglais | LILACS | ID: lil-604150
ABSTRACT
A 12-year-old boy with difficulty in wound healing and scars of unusual appearance was examined. Thin, shiny and protruding skin, characterizing pseudotumoral lesions, was observed on his knees and elbows. Loose joints and increased skin elasticity were also observed. Light microscopy showed rare collagen bundles and loosely dispersed collagen fibers. Irregularly distributed elastic tissue was observed, and transmission electron microscopy revealed disorganized collagen fibers. In cross sections under high magnifications, fibers of varying diameters could be seen, as well as an irregular fiber outline. Scanning electron microscopy of the dermis showed disorganized collagen fibers, which were rarely arranged in bundles. Isolated and crossed-over fibers were also found.
RESUMO
Paciente masculino de 12 anos relatou dificuldade em cicatrizar, seguido por cicatrizes de aspecto infrequente . A pele dos joelhos e cotovelos é fina, brilhante e protrusa, caracterizando pseudo- tumores. Além disso apresenta hiperelasticidade cutânea e articular. A microscopia óptica demonstrou raros feixes colágenos, as fibras colágenas encontram-se dispersas. O tecido elástico está presente e secundariamente irregular. A microscopia eletrônica de transmissão também observou fibras colágenas desorganizadas e com cortes transversais em grande aumento evidenciou diâmetros variados e contorno irregular das mesmas. A microscopia eletrônica de varredura da derme demonstrou raros feixes colágenos e fibras colágenas isoladas e entrecruzadas.
Sujets)


Texte intégral: Disponible Indice: LILAS (Amériques) Sujet Principal: Peau / Syndrome d'Ehlers-Danlos Limites du sujet: Enfant / Humains / Mâle langue: Anglais Texte intégral: An. bras. dermatol Thème du journal: Dermatologie Année: 2011 Type: Article Pays d'affiliation: Brésil Institution/Pays d'affiliation: Catholic University of Pelotas/BR / EMBRAPA/BR / Santa Casa de Misericórdia Porto Alegre/BR

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Texte intégral: Disponible Indice: LILAS (Amériques) Sujet Principal: Peau / Syndrome d'Ehlers-Danlos Limites du sujet: Enfant / Humains / Mâle langue: Anglais Texte intégral: An. bras. dermatol Thème du journal: Dermatologie Année: 2011 Type: Article Pays d'affiliation: Brésil Institution/Pays d'affiliation: Catholic University of Pelotas/BR / EMBRAPA/BR / Santa Casa de Misericórdia Porto Alegre/BR