Taylor G, Venning V, Wojnarowska F, Millard P R
Department of Dermatology, Slade Hospital, Oxford, U.K.
J Cutan Pathol. 1993 Oct;20(5):389-92. doi: 10.1111/j.1600-0560.1993.tb00659.x.
In the Weber-Cockayne form of epidermolysis bullosa simplex (EBS-WC), trauma induces blisters which are confined to the palms and soles. Histologically, basal cell cytolysis is seen. We studied 6 patients with EBS-WC to determine the ultrastructural level at which artificially-induced suction blisters form. Blisters were raised by application of a suction blister cup to uninvolved forearm skin, the cup being connected to a negative pressure of 200 mm of mercury. The blisters were biopsied and examined by light and electron microscopy. On light microscopy, all biopsies showed marked vacuolization of keratinocytes in the lower two-thirds of the epidermis, and in all but one there was a cleavage plane through the basal keratinocytes. These findings were confirmed by electron microscopy in 4 patients. The separation through the basal cells is in contrast to the situation in normal individuals in whom cleavage occurs below the level of the basal cells, within the lamina lucida. Thus, even apparently normal skin from non-acral sites has the same structural abnormality as the affected acral sites in EBS-WC.
在单纯性大疱性表皮松解症的Weber-Cockayne型（EBS-WC）中，外伤会引发水疱，且水疱局限于手掌和脚底。组织学上，可见基底细胞溶解。我们研究了6例EBS-WC患者，以确定人工诱导的抽吸性水疱形成的超微结构水平。通过将抽吸性水疱杯应用于未受累的前臂皮肤来产生水疱，该水疱杯连接到200毫米汞柱的负压。对水疱进行活检，并通过光学显微镜和电子显微镜检查。光学显微镜下，所有活检标本均显示表皮下三分之二的角质形成细胞有明显空泡化，除1例之外，其余所有标本在基底角质形成细胞处均有一个分裂平面。4例患者的这些发现通过电子显微镜得到证实。通过基底细胞的分离与正常个体的情况相反，正常个体的分裂发生在基底细胞水平以下、透明层内。因此，即使是来自非肢端部位的看似正常的皮肤，其结构异常也与EBS-WC中受影响的肢端部位相同。
