Extensive Fibrin Accumulation and Accompanying Epithelial Changes in the Pathogenesis of Ligneous Mucosal Disease (Ligneous Periodontitis)

Abstract

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.

Immunohistochemical analysis of the gingiva with periodontitis of type I plasminogen deficiency compared to gingiva with gingivitis and periodontitis and healthy gingiva

“…To date there have been many case reports reporting ligneous gingival lesions (El Darouti et al, 2009;Fine, Bauer, Al Mohaya, & Woo, 2009;Galeotti et al, 2014;Gokbuget et al, 1997;Gunhan et al, 1994Gunhan et al, , 1999Gunhan et al, 2012;Kurtulus et al, 2007;Scully et al, 2001;Silva, Bariani, Mendonca, & Batista, 2006;Suresh et al, 2003;Toker, Toker, Goze, Turgut, & Yilmaz, 2007), however, as far as we know, the possible role that plg may play in the maintenance of periodontal health has been first studied by Sulniute et al (2011) in mice. The authors were the first to show that the development of periodontitis in mice resembles that in humans and is characterized by the formation of necrotic tissue, the inflow of neutrophils, detachment of gingival tissue from the teeth, followed by severe alveolar bone resorption (Sulniute et al, 2011).…”

“…Ligneous lesions of the oral mucosa have been histopathologically described extensively (El Darouti, Zayed, El Kamah, & Mostafa, 2009; Gokbuget et al, 1997;Gunhan et al, 1994;Gunhan, Avci, Dereci, Akgun, & Celasun, 2012;Scully et al, 2001Scully et al, , 2007. According to this, gingival tissues from the 8 patients in our report were examined and diagnosed with ligneous periodontitis.…”

Ligneous Periodontitis in a Patient with Type 1 Plasminogen Deficiency: A Case Report and Review of the Literature

“…The exact pathophysiology of the clinical presentation seen in LP is still not clear. In vitro data and animal studies indicated that alterations in tissue repair and host defense mechanisms are responsible for the onset and the progression of periodontal destruction [4,26]. Plasminogen plays an important role in intravascular and extravascular fibrinolysis, wound healing, cell migration, tissue remodelling, angiogenesis, and embryogenesis [27].…”

Periodontitis associated with plasminogen deficiency: a case report

“…The exact pathophysiology of ligneous periodontitis remains, however, unclear [ 18 , 1 ]. In vitro data and animal studies indicated that alterations in tissue repair and host defense mechanisms are responsible for the onset and the progression of periodontal destruction [ 1 , 19 ]. Local extracellular fibrinolysis by plasmin is required for the initial removal of the fibrin-rich matrix as well as for the remodeling of the granulation tissue and completion of wound healing [ 9 , 20 , 5 ].…”