Bruckner-Tuderman L, Niemi K M, Kero M, Schnyder U W, Reunala T
Department of Dermatology, University of Zurich, Switzerland.
Br J Dermatol. 1990 Mar;122(3):383-90. doi: 10.1111/j.1365-2133.1990.tb08287.x.
A patient with dystrophic epidermolysis bullosa inversa was studied using electron microscopy and indirect immunofluorescence using antibodies to matrix macromolecules of the dermoepidermal junction zone. There was splitting below the lamina densa with an apparently normal basement membrane, but a lack of intact anchoring fibrils and with a disarranged papillary connective tissue. Indirect immunofluorescence examination with antibodies to type VII collagen, the major structural protein of anchoring fibrils, showed a normal linear staining pattern. Synthesis of type VII collagen which is unable to form stable, resistant anchoring fibrils may be a distinct feature of this subtype of recessive dystrophic epidermolysis bullosa.
利用电子显微镜和针对真皮表皮交界区基质大分子的抗体进行间接免疫荧光，对一名反向营养不良性大疱性表皮松解症患者进行了研究。在致密板下方出现分裂，基底膜明显正常，但缺乏完整的锚原纤维，乳头结缔组织排列紊乱。用针对锚原纤维的主要结构蛋白VII型胶原的抗体进行间接免疫荧光检查，显示出正常的线性染色模式。无法形成稳定、抗降解的锚原纤维的VII型胶原的合成可能是这种隐性营养不良性大疱性表皮松解症亚型的一个独特特征。
