Classical Ehlers-Danlos syndrome: clinical, Histological and ultrastructural aspects

Anais Brasileiros de Dermatologia

Eduardo De Barros Coelho Bicca²  Fabiano Bonow De Almeida²  Giselle Martins Pinto¹  Luis Antônio Suíta De Castro¹  Hiram Larangeira De Almeida Jr² 

[1] ,Catholic University of PelotasPelotas RS ,Brazil

关键词: Collagen type V;    Ehlers-Danlos syndrome;    Microscopy;    electron;    scanning;    Microscopy;    electron;    scanning transmission;    Colágeno tipo V;    Microscopia eletrônica de transmissão;    Microscopia eletrônica de varredura;    Síndrome de Ehlers-Danlos;   

DOI  :  10.1590/S0365-05962011000700043

来源: SciELO

【 摘 要 】

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

【 授权许可】

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