Günhan Ömer, Avci Arzu, Dereci Ömür, Akgün Sivge, Celasun Bülent
Department of Pathology, Gülhane Military Medical Academy, Ankara, Turkey.
Am J Dermatopathol. 2012 Feb;34(1):35-40. doi: 10.1097/DAD.0b013e3182169507.
Certain abnormal products of human tissues are resistant to degradation. The fibrillary ultrastructure of some of these are seen integrated with normal tissue components. The accumulations seen in colloid milium, lichen, and macular amyloidosis are of this type. Apoptosis of keratinocytes and filamentous degeneration of some proteins can be important in the pathogenesis. A similar pathogenetic mechanism is possible in ligneous mucosal disease, which is a rare disorder of plasminogen deficiency characterized by amyloid-like amorphous accumulations. Gingival and conjunctival mucosal pseudomembraneous masses are typical and concomitant involvement of other sites are not unusual. The accumulated substance is thought to be an abnormal fibrin degradation product. In this study, we have examined 6 representative samples from 5 gingival and 1 conjunctival lesions displaying characteristic features. Immunohistochemically, fibrinogen was detected as an early change. TUNEL staining revealed numerous apoptotic keratinocytes in this phase as well. These cells also expressed nuclear factor kappa beta. Apoptotic cells showed loss of epithelial cadherin immunostaining. In the later phase, the subepithelial accumulations failed to stain with antifibrinogen, wide spectrum, and high molecular keratins, type 4 collagen and nuclear factor kappa beta. Our findings suggest that the accumulations in ligneous mucosal disorder result from an abnormal healing process and they probably form as a combination of organised fibrinogen, epithelial fragments, and connective tissue matrix.
人体组织的某些异常产物具有抗降解性。其中一些产物的纤维超微结构可见与正常组织成分整合在一起。见于胶样粟丘疹、扁平苔藓和黄斑淀粉样变的沉积物即属此类。角质形成细胞的凋亡和某些蛋白质的丝状变性在发病机制中可能起重要作用。在木样黏膜病中可能存在类似的发病机制，木样黏膜病是一种罕见的纤溶酶原缺乏症，其特征为淀粉样无定形沉积物。牙龈和结膜黏膜假膜性肿物很典型，其他部位同时受累也并不罕见。积聚的物质被认为是一种异常的纤维蛋白降解产物。在本研究中，我们检查了来自5例牙龈病变和1例结膜病变的6个具有特征性表现的代表性样本。免疫组织化学检测显示，纤维蛋白原是早期变化。TUNEL染色在这个阶段也显示出大量凋亡的角质形成细胞。这些细胞还表达核因子κB。凋亡细胞显示上皮钙黏蛋白免疫染色缺失。在后期，上皮下沉积物不能被抗纤维蛋白原、广谱和高分子角蛋白、4型胶原蛋白以及核因子κB染色。我们的研究结果表明，木样黏膜病中的沉积物是由异常愈合过程导致的，它们可能是由有组织的纤维蛋白原、上皮碎片和结缔组织基质组合而成。
