Oral lesions indicative of plasminogen deficiency (hypoplasminogenemia).

BACKGROUND: Gingival overgrowth with ulceration has recently been recorded in 4 reports: (1) our report of a British patient with ligneous conjunctivitis in whom the gingival lesions appeared to be related to tranexamic acid-an antifibrinolytic agent; (2) a report of 2 Turkish patients and an Italian patient with mainly gingival lesions; (3) our report of 5 Turkish patients with mainly gingival lesions; and (4) a report of 3 new Turkish cases, which also were associated with gingival lesions and alveolar bone loss. These patients all had gingival swellings, and a minority had conjunctival involvement similar to ligneous conjunctivitis, although the etiology was unclear in all. Nevertheless, fibrin exudation was fundamental because the hyaline or amyloidaceous material seen on the gingival biopsy stained for fibrin but failed to stain for amyloid. METHODS: We have examined 6 more patients who exhibited gingival swelling caused by amyloidaceous deposits that stained only for fibrin, and we assayed their plasminogen levels. RESULTS: The plasminogen functional activity assayed in these 6 additional patients, and in 2 of the 5 patients previously reported by us, was significantly reduced. CONCLUSIONS: Gingival overgrowth with ulceration appears to be a new complication caused by plasminogen deficiency; it also appears to be related to ligneous conjunctivitis in some cases.

Sublocalization of the Papillon-Lefevre syndrome locus on 11q14-q21.

Papillon-Lefevre syndrome (PLS) is an autosomal recessive form of palmoplantar ectodermal dysplasia, characterized by palmoplantar hyperkeratosis and severe early-onset periodontitis. The presence of severe periodontitis distinguishes PLS from other palmoplantar keratodermas. As part of our efforts to study the genetic basis of periodontitis susceptibility, we performed a genome-wide search to identify major loci for PLS in 44 individuals (14 affected) from 10 consanguineous PLS families. We have identified evidence for linkage of a PLS gene on 11q14-q21. A maximum two-point logarithm of the odds (LOD) score of 8.24 was obtained for D11S1367 at a recombination fraction of theta=0.00. Multipoint analysis resulted in a LOD score of 10.45 and placed the gene for PLS within a 4-5 cM genetic interval. This genetic interval, flanked by D11S4197 and D11S931, contains more than 50 cDNAs and 200 expressed sequence tags (ESTs). This refinement of the candidate region for a PLS gene is in agreement with other recent reports of linkage for PLS to chromosome 11q14-q21 and should help in identification of the gene for PLS.

Amyloidaceous ulcerated gingival hyperplasia: a newly described entity related to ligneous conjunctivitis.

Gingival hyperplasia may be genetic, may be acquired as a consequence of exposure to drugs and other agents or may appear as part of a more widespread disorder. Five patients who acquired gingival hyperplasia due to amyloidaceous deposits staining only for fibrin are presented. This appears to be a new entity related to ligneous conjunctivitis.

Generalized prepubertal periodontitis. A report of 4 cases with the immunological findings.

The clinical and immunological data from 4 patients with generalized prepubertal periodontitis are presented. The peripheral blood neutrophil chemotaxis was measured using zymosan activated sera as the chemoattractant. All of the 4 patients have shown depressed neutrophil chemotaxis compared to those of the healthy controls. Peripheral blood lymphocyte subpopulations were analysed by double-coloured flow cytometry using monoclonal antibodies for the receptors CD2, CD3, CD4, CD8, CD19, CD29, CD45RA+, 34,29dK, CD56. CD11b/CD18. Lymphocytes bearing CD3 receptors showed a significant decrease compared to those of the controls. Natural killer cells were lowered in 3 of the 4 cases. All of the patients showed a higher increase in CD11b/CD18 expression. The evaluation of CD11b/CD18 receptor in peripheral blood leukocytes may be of help explaining the rôle of neutrophils in the pathogenesis of the disease.

Clinical and immunological findings in 2 siblings with Papillon-Lefèvre syndrome.

Rapid and severe destruction of periodontal tissues in early childhood has been reported both in systemically healthy children and in children with systemic disorders. In this study, the clinical and immunological findings of two siblings in a family with Papillon-Lefèvre syndrome are presented. The peripheral blood lymphocytes were analyzed using a double colored flow cytometry and adequate monoclonal antibodies to CD2, CD3, CD4, CD5, CD8, CD11b, CD16, CD19, and HLA-DR receptors. CD11b expression was found to be higher in both siblings (35% and 37%). The elevated CD11b expression may be related to a defect in neutrophils. The expression of natural killer cells was found to be higher in one patient but the results were in normal range. The CD2+, CD3+, CD4+, CD5+, CD8+, and CD19+ lymphocytes were in normal range in both patients. We think that the depressed chemotaxis of peripheral neutrophils, and higher expression of HLA-DR and CD11b molecules in peripheral leukocytes were useful in explaining the pathogenesis of the Papillon-Lefèvre syndrome.

Papillon-Lefèvre syndrome. Analysis of peripheral blood lymphocyte subsets.

We have studied the peripheral blood lymphocyte populations in our 6 patients (2 female and 4 male) with a mean age of 11.19 with Papillon-Lèfevre Syndrome (PLS) using adequate monoclonal antibodies and double coloured flow cytometry. Total B, T, CD4, CD8, CD29, CD45RA, NK, HLA-DR cells were studied. Total B, T, CD4 and CD8 lymphocytes were within normal limits. We have observed an increase in the CD29 lymphocytes and NK cells and a decrease in CD45RA lymphocytes. We think that these findings are important in explaining B lymphocyte activation and in the pathogenesis of the PLS.

Papillon-Lefèvre syndrome. Analysis of neutrophil chemotaxis.

Papillon-lefèvre syndrome (PLS) is described as the association of palmar-plantar hyperkeratosis with precocious periodontal disease which results in exfoliation of primary and permanent dentitions. This study was planned to assess the chemotaxis of peripheral blood neutrophils in 7 patients (3 females and 4 males) with Papillon-Lefevre syndrome. The neutrophil chemotaxis was analyzed using the zymosan activated serum (ZAS) assay. Chemotaxis and spontaneous migration measurements were compared to those of the healthy control subjects. The peripheral blood neutrophil chemotaxis and spontaneous migration were depressed in all patients with Papillon-Lefèvre syndrome. The decreased chemotaxis of peripheral blood neutrophils strongly suggests that the neutrophils may act as one of the important key determinants in the pathogenesis of severe periodontal destruction in patients with PLS.

Clinical evaluation of the effect of tetracycline hydrochloride root conditioning during flap surgery.

Clinical effects of root surface demineralization during flap surgery were evaluated in ten patients with rapidly progressive periodontitis. Following initial periodontal therapy, flap surgery was conducted on the anterior segments. Test sites received conditioning with tetracycline solution for 5 minutes, whereas control sites were only rinsed with sterile saline solution. Following either treatment, improvement was observed on all clinical measures. However, apart from gingival index assessments, there was not a statistically significant difference in the improvement observed between test and control groups; the only significant difference was observed in gingival index scores at the second and fourth weeks. These differences in gingival index, which are not reflected in plaque index scores, imply that tetracycline has been absorbed on root surfaces and released in biologically active form for up to 4 weeks; but attachment level measurements demonstrate that tetracycline conditioning of the root surfaces during flap surgery has no additional regenerative benefit, or at least none detectable by clinical means.

A further report of bony exostosis occurring as a sequela to free gingival grafts.

Two cases of bony exostosis developing at the site of previously placed free gingival grafts are presented. It is suggested that the etiology is surgical trauma.