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

Abstract

Background. Ligneous periodontitis or destructive membranous periodontal disease is a rare condition involving gingival tissues, which is due to plasminogen deficiency and fibrin deposition. Plasminogen deficiency is an ultrarare autosomal recessive disease. The disease is characterized by gingival enlargement and periodontal tissue destruction that leads to rapid tooth loss despite treatment attempts. A defect in fibrinolysis and abnormal wound healing are the main pathogenesis of this condition. It is caused by mutations in PLG, the gene coding for plasminogen, which results in decreased levels and functional activity. Case Presentation. In this case report, clinical and histopathological findings of a 26-year-old male patient who presented with generalized membranous gingival enlargement are presented. He was the third child of consanguineous parents and had multicystic congenital hydrocephalus at birth. Besides the gingival enlargement, he also presented ligneous conjunctivitis since childhood. The intraoral examination revealed generalized periodontal breakdown. Radiographs showed alveolar bone loss present in every quadrant. All blood investigations were normal except for plasminogen deficiency. A biopsy sample was excised from affected gingiva and a series of histopathological evaluation was performed. Based on clinical and histopathological evidence, a diagnosis of destructive membranous periodontal disease or ligneous periodontitis was made. A clinical exome assay for the PLG gene was also done. It was confirmed as Type 1 plasminogen deficiency. Conclusion. Ligneous periodontitis has been rarely reported in India. The reasons could be because of the rarity of the disease or missed diagnosis. The need to take a proper history and perform a proper clinical examination and histopathologic evaluation has to be stressed when diagnosing and treating gingival enlargements. If a genetic condition is suspected, genetic screening is also needed. All these will help the clinician in correctly diagnosing the disease and formulating a proper treatment plan for managing the condition.

Ligneous Gingivitis: A Systematic Review

“…Searches identified a total 59 papers, and after final analysis according to the inclusion criteria 27 papers were acceptable (Figure 1). There were 21 case reports [10,[14][15][16][17][18][19][20][21]23,24,26,27,[30][31][32][33][38][39][40][41], and six were case series [1,2,8,9,34,35] Case report 1 3a Turkey Pierro VS 17 Case report 1 3a Brazil Suresh L 39 Case report 1 3a USA Günhan O 9 Case series 3 3a Turkey Gokbuget AY 1 Case series 5 3b Turkey Fine G 16 Case report 1 3a USA Baykul T 27 Case report 1 4 Turkey Chi AC 14 Case…”

“…Concerning race there was a predominance of white patients with 63.3% (n=31) cases, principally from Turkish descendants in 53.1% (n=26) of cases, although in that country there a lot of races, the literature mentioned there were white, however, it is important to mention that in 30.6% (n=15) cases, race was not reported. For this reason, publication bias could increase "white" race for the patients reported and may not reflect true incidence of oral LG, due to black cases were only 6.1% (n=3), because LG cases demonstrated a worldwide distribution, which in our study were from 14 countries [10][11][12][13][14][15][16][17]20,23,24]. Regarding symptoms, pain was the most common with 40.8% (n=20) cases, similar to the result by Klammt J et al, in a series of 23 cases with low PLG activity [7], followed by a painless lesion in 20.4% (n=10) of cases.…”

“…Concerning the clinical presentation of 33 cases reported in the gingiva, describing the oral lesions for LG "gingival irregular yellow-white overgrowth" was the most common description with 40.8% (n=20) cases [1,8,9,[15][16][17]21,23,27,39,41], followed by "gingival ulcer mass", [1,2,10,18,19,24,26,31,34,38] with 30.6% (n=15) cases. The description of gingival enlargements covered by yellow white pseudomembranes was more prevalent for gingival overgrowth, than for gingival ulcer mass.…”

Delayed-onset ligneous conjunctivitis as a rare association with congenital hydrocephalous: a case report and review of the literature

“…The first presentation of ligneous conjunctivitis was coincidental with hydrocephalous in infancy or childhood, with a short period between presentation of both conditions. 1,4,[13][14][15][16][17][18][19][20] We found no other report of delayed-onset ligneous conjunctivitis associated with congenital hydrocephalus.…”

Rare Genetic Disorders Affecting the Periodontal Supporting Tissues in Adolescence

“…Plasminogen is not only a complement inhibitor but also a broad-spectrum proteolytic factor either by directly degrading extracellular matrix proteins, e.g., laminin, fibronectin and proteoglycans, and indirectly by activating latent metalloproteinases (39). Plasminogen deficiency is associated with massive fibrin deposition within mucous membranes due to the absence of fibrin clearance by plasmin, leading to a so-called "ligneous" conjunctivitis (80% of patients), ligneous gingivitis (34%) and ligneous periodontitis, characterized by extensive generalized periodontal breakdown in association with pseudomembranous gingival enlargement (12)(13)(14)(15). The diagnosis is confirmed by the identification of biallelic pathogenic PLG mutations in molecular genetic analysis.…”