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科凯恩-图赖讷型显性营养不良性大疱性表皮松解症——与帕西尼变异型的超微结构相似性。

The Cockayne-Touraine type of dominant dystrophic epidermolysis bullosa--ultrastructural similarities to the Pasini variant.

作者信息

Oakley C A, Gawkrodger D J, Ross J A, Hunter J A

出版信息

Acta Derm Venereol. 1984;64(3):253-6.

PMID:6204490
Abstract

Biopsies from the uninvolved skin of 5 patients with the Cockayne-Touraine (CT) type of dystrophic epidermolysis bullosa (DEB) were studied with the electron microscope. Dermal fibrillar bodies were noted in 2 patients and 3 showed basal lamina (BL) duplication or splitting. Discontinuity of the BL with herniation of keratinocyte cytoplasm was present in one patient. These changes, thought previously to be characteristic of the Pasini variant, indicate that abnormalities of the BL may be involved in blister formation in both disease subgroups.

摘要

对5例患有科凯恩-图赖讷(CT)型营养不良性大疱性表皮松解症(DEB)患者未受累皮肤进行活检,并用电镜进行研究。在2例患者中发现了真皮纤维状体,3例显示基底膜(BL)重复或分裂。1例患者存在基底膜连续性中断并伴有角质形成细胞胞质疝出。这些变化以前被认为是帕西尼变种的特征,表明基底膜异常可能与两个疾病亚组的水疱形成有关。

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