EVALUATION OF ANCHORING FIBRILS AND OTHER COMPONENTS OF THE DERMAL-EPIDERMAL JUNCTION IN DYSTROPHIC EPIDERMOLYSIS BULLOSA BY A QUANTITATIVE ULTRASTRUCTURAL TECHNIQUE
EVALUATION OF ANCHORING FIBRILS AND OTHER COMPONENTS OF THE DERMAL-EPIDERMAL JUNCTION IN DYSTROPHIC EPIDERMOLYSIS BULLOSA BY A QUANTITATIVE ULTRASTRUCTURAL TECHNIQUE
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DOI：
10.1111/1523-1747.ep12265460
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发表时间：
1985-01-01
影响因子：
6.5
通讯作者：
EADY, RAJ
中科院分区：
文献类型：
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作者：
TIDMAN, MJ;EADY, RAJ
大疱性营养不良性表皮松解症(DEB)患者真皮-表皮交界处锚定纤维(AF)和其他成分的结构和群体密度的差异可能有助于区分遗传和临床上不同类型的DEB，因此对17例DEB患者进行了未分离的角质形成细胞相关真皮-表皮交界处的超微结构对照研究。7例为显性DEB，3例为局限性隐性DEB，7例为重度全身性隐性DEB。无皮损、无疤痕的皮肤取自标准的身体部位。房颤的诊断标准是必须与致密层结合，并存在四肢中央环扎和/或扇形。在9例符合技术条件的重度隐性DEB患者标本中未检测到房颤。结构上正常的房颤在显性和局部性隐性DEB中都存在，但与来自12名健康成年人的位点匹配的样本相比，数量显著减少。显性和局部性隐性DEB之间、水泡好发部位和非易发部位之间的房颤特征无明显差异。优势DEB白球样病变的存在或不存在不影响房颤的计数。与对照组相比，DEB患者组的半桥体、基底细胞质膜小泡或真皮微纤维束的数量均无明显差异。虽然常规的透射电子显微镜可以区分严重毁损的DEB，但不能根据AF的结构或数目来区分显性和局部性隐性形式。
Differences in the structure and population density of anchoring fibrils (AF) and other components of the dermal-epidermal junction might distinguish between genetically and clinically distinct varieties of dystrophic epidermolysis bullosa (DEB), so a controlled ultrastructural morphometric study of nonseparated keratinocyte-associated dermal-epidermal junction was undertaken in a total of 17 patients with DEB. Seven patients had dominant DEB, 3 had localized recessive DEB and 7 had severe, generalized recessive DEB. Nonlesional, unscarred skin was obtained from standard body regions. Criteria for the identification of AF were a mandatory union with the lamina densa and the presence of central banding and/or fanning of the extremities. No AF were detected in 9 technically suitable samples from patients with severe recessive DEB. Structurally normal AF were present, but significantly reduced in number, in both dominant and localized recessive DEB, compared with site-matched samples from 12 healthy adults. There was no difference in AF characteristics between dominant and localized recessive DEB, or between sites of predilection and nonpredilection for blisters. The presence or absence of albopapuloid lesions in dominant DEB did not influence AF counts. There was no difference in numbers of hemidesmosomes, basal cell plasmalemmal vesicles, or dermal microfibril bundles in any group of DEB patients compared with controls. Although severe mutilating DEB can be distinguished by routine transmission electron microscopy, the dominant and localized recessive forms cannot be differentiated on the basis of AF structure or numbers.