Papillon-Lefèvre Syndrome Patients Research Articles

Self-reported clinical features and treatment effectiveness of Papillon-Lefèvre syndrome patients from five Latin American countries: A cross-sectional online survey study.

Most studies about Papillon-Lefèvre syndrome (PLS) are limited to case reports and patients of the same nationality. This study aimed to determine the self-reported prevalence of signs, symptoms and treatment effectiveness in PLS patients from five Latin American countries. An online survey was conducted among adult and paediatric patients from Mexico, Argentina, Colombia and Brazil. Data were collected using multiple-choice, open-ended and image-chooser questions on demographics, signs and symptoms, perceived treatment effectiveness and quality of life. Seventeen patients (10 males and 7 females) aged 4-47 years were surveyed. All had palmoplantar hyperkeratosis. Other affected sites were the feet and hand dorsum (82.35%), Achilles tendon (88.24%), forearms (58.82%), legs (29.41%) and glutes (23.53%). They frequently presented hyperhidrosis and nail pitting. Four had a history of delayed umbilical cord separation. All used topical treatments, with moderate effectiveness; half used oral retinoids, perceived as highly effective. Most reported decreased quality of life and walking difficulties. The study's results align with prior research on PLS, but reveal new insights, including the impact on patients' quality of life and a history of delayed umbilical cord separation. These findings warrant consideration in future research and patient care.

Recent developments in the diagnosis, treatment, and management of Papillon-Lefèvre Syndrome.

A systematic search of PubMed, LIVIVO, and Ovid was conducted up to March 2021. These databases were searched for relevant clinical studies on periodontal treatment success in individuals with Papillon-Lefèvre syndrome (PLS). Clinical studies reporting successful treatment outcomes defined as the loss of four or fewer permanent teeth due to periodontitis and the arrest of periodontitis or probing depths of 5 mm or less in individuals with PLS followed up for ≥24 months were included, and data extracted. Twelve studies reporting on nine PLS patients met the inclusion criteria. The extracted main outcomes in the studies reporting successful periodontal treatment in PLS were as follows: (1) clinical and genetic diagnosis of PLS; (2) age at baseline; (3) initial dental, periodontal parameters, and microbiological assessment, if available; (4) description of disease progression and applied therapies; and (5) outcome and follow-up. Twelve studies reporting nine individuals were included. The timely extraction of affected or all primary teeth, compliance with oral hygiene instructions, supra- and subgingival debridement within frequent supportive periodontal care intervals, and adjunctive systemic antibiotic therapy in most patients affected a halt in disease progression. Suppression of Aggregatibacter actinomycetemcomitans below detection limits was associated with periodontal stabilization. An intensive, multidisciplinary approach with strict compliance may enable the decelerated progression of PLS-associated periodontitis. The early diagnosis of PLS and the suppression of A. actinomycetemcomitans below the detection level might be critical factors for treatment success. It required significant effort and patient compliance. The study emphasized the importance of timely interventions, oral hygiene maintenance, regular professional dental care, and, in some cases, systemic antibiotics.

Oral Manifestation of Papillon-Lefevre in Two Siblings

Background: Papillon-Lefèvre Syndrome (PLS) is a very rare genetic disorder (autosomal recessive) that usually affects infants between the ages of one and five years. PLS is characterized by palmar-plantar hyperkeratosis and premature loss of primary and permanent teeth with extensive, severe, and rapid destruction of the alveolar bone, leading to the premature loss of both teeth. Objective: To report on the management of PLS patients. Case: A 9-year-old girl and a 2.5-year-old boy from Nganjukto General Hospital, Pediatric Dentistry Division, RSGM Airlangga University with the main complaints of upper left tooth pain, rocking, difficulty eating, erythematous hyperkeratosis hands and feet, periodontal inflammation, mobility. bad teeth. Case Management: Nine-year-old girl: Teeth #26 and #46 could not be retained and extracted due to buccoversion and severe mobility. Periodontal supportive therapy was periodontal debridement, scaling and root planing and oral hygiene counseling with 0.20% chlorhexidine gluconate. Two-and-a-half-year-old boy: Too young, he is observed every 3 months. It is recommended to take vitamin B complex in appropriate doses and fruit juice as an additional supplement. Conclusion: Intensive periodontal treatment and administration of antibiotics only delays the progression of periodontal disease and cannot be used to prevent primary and permanent teeth. Extensive and repeated prosthodontic treatment is required to provide functional teeth to children during their period of jaw growth.

Abnormal profiles of cathepsin C secreted in urine of Papillon Lefevre syndrome patients

BackgroundPapillon Lefevre syndrome (PLS) is an autosomal recessive disorder that results from a mutated gene that encodes a lysosomal peptidase known as cathepsin C (CTSC). The clinical presentation of PLS involves mainly palmoplantar keratosis and periodontitis with a variable degree of severity. Subjectsand methods: Our study included ten patients with a broad spectrum of palmoplantar keratosis and periodontitis severity. CTSC variants were detected by Sanger sequencing. CTSC protein secreted in urine was detected by western blotting. ResultsFive patients have missense variants, Four have nonsense variants, and one has splice variants in CTSC. The activation products of cathepsin C protein (Heavy and light chains) were absent in all patients’ urine samples except one with a significantly reduced level compared to the controls. The dimeric form of CTSC protein was found in all the studied cases. The monomeric form was found in five cases. The products of proteolytic activation of CTSC by other cathepsins (L and S) were found in the urine samples of five of the patients. Each patient had a characteristic pattern of accumulated CTSC protein maturation/activation substrates, intermediates, and products. 40% of the patients had the activation products of other lysosomal cathepsins. ConclusionUrinary CTSC in PLS patients could be used as a diagnostic biomarker for the biochemical screening of the disease. Different variants in CTSC result in different profiles of CTSC secreted in the urine of PLS patients. The profiles of secreted CTSC in urine could be correlated to the severity of palmoplantar keratosis.

Papillon-LefÈvre Syndrome: Report of Two Cases in Siblings

<h3>Introduction</h3> Papillon-Lefèvre syndrome (PLS) is an unusual health condition manifested by pre-pubertal extensive periodontal disease that results in early tooth loss and palmoplantar keratosis. The etiology of PLS is loss-offunction mutations in the cathepsin C gene. This gene is a lysosomal protease that plays an essential role in immune and inflammatory responses. <h3>Case Presentation</h3> We present two cases in siblings with PLS. The parents of the patients are relative. The first patient is 20 years old who presented to the clinic with highly resorbed maxillary and mandibular alveolar ridges with only wisdom teeth remaining in the oral cavity at the time of examination. According to the dental history, there was aggressive periodontitis followed by complete destruction of the periodontium and severe bone resorption. There was skin manifestations of PLS including palmoplantar and dorsal keratosis in their extremities as well as the knee and wrist joint regions. The second sibling was 9 years old with only skin manifestations similar to the older sibling. The periodontal status of the younger patient was stable at the time of clinical examination. Interestingly, they reported that their 16-year-old female cousin also suffers from the same condition, with almost the same clinical manifestations as the older sibling. <h3>Conclusion</h3> We report two cases of PLS in patients of same family, which supports the hereditary nature of cathepsin C mutation in PLS patients. In addition, variability of clinical expression of PLS is very evident in the current cases, which reflects that the PLS can show a broad spectrum of clinical features.

Survival Rates of Dental Implants in Patients with Papillon&amp;ndash;Lef&amp;eacute;vre Syndrome: A Systematic Review

The present review aimed to summarize and evaluate the available literature regarding the survival rate and outcomes of dental implants in patients with Papillon-Lefèvre syndrome (PLS). An extensive search of the literature was conducted on PubMed, Scopus and Web of Science databases for all data published from January 1996 till April 2020 using a combination of the following keywords: 'Papillon Lefévre Syndrome', 'prosthodontic rehabilitation' and 'dental implant' according to the PRISMA guidelines for the focused research question constructed using the PICO criteria. Clinical trials and observational studies on implant placement in PLS patients reported in English language were included in the study. A total of 10 studies (nine case reports and one case series) comprising 124 dental implants placed in 13 PLS patients were included. The follow-up period ranged from 4 months to 9 years. With regard to implant loading, 9 studies reported delayed loading, while one study did not provide any information regarding the nature of implant loading. The design of prosthodontic superstructure was either a removable or fixed prosthesis. Out of the 124 inserted implants, 20 (16%) were reported as failed. The overall survival rate was 84%. The limited available evidence suggests that the survival rate of dental implants in patients with PLS is lower than that among healthy individuals. Nevertheless, no strict contraindication for implant-supported prosthesis seems to be justified in this group of patients. Further longitudinal studies with adequate follow-up periods are highly warranted. The prognosis of implant treatment for PLS patients has not yet been established. Dental practitioners should follow a careful approach in planning the dental implant treatment for this cohort of patients.

Potential Role of the Mitochondria for the Dermatological Treatment of Papillon-Lefèvre

The Papillon–Lefèvre syndrome (PLS) is a rare autosomal recessive disorder caused by mutations in the Cathepsin C (CTSC) gene, characterized by periodontitis and palmoplantar hyperkeratosis. The main inflammatory deficiencies include oxidative stress and autophagic dysfunction. Mitochondria are the main source of reactive oxygen species; their impaired function is related to skin diseases and periodontitis. The mitochondrial function has been evaluated in PLS and mitochondria have been targeted as a possible treatment for PLS. We show for the first time an important mitochondrial dysfunction associated with increased oxidative damage of mtDNA, reduced CoQ10 and mitochondrial mass and aberrant morphologies of the mitochondria in PLS patients. Mitochondrial dysfunction, determined by oxygen consumption rate (OCR) in PLS fibroblasts, was treated with CoQ10 supplementation, which determined an improvement in OCR and a remission of skin damage in a patient receiving a topical administration of a cream enriched with CoQ10 0.1%. We provide the first evidence of the role of mitochondrial dysfunction and CoQ10 deficiency in the pathophysiology of PLS and a future therapeutic option for PLS.

Nonsurgical periodontal management of Papillon–Lefevre syndrome

Papillon–Lefevre syndrome (PLS) is an autosomal recessive genetic disorder caused by a deficiency in Cathepsin C. It is characterized by palmoplantar keratosis and premature loss of both primary and permanent dentition. Patients are often edentulous at an early age. Other symptoms include increased susceptibility to infections. Various etiologic factors such as genetic mutations, immunologic alterations, and bacteria have been implicated. We present a case report of 12-year-old female who reported to the dental clinic with a history of pain and swollen gums. Family history revealed consanguinity. On clinical examination, there was palmoplantar keratosis and examination of the oral cavity showed generalized periodontal pockets, tooth mobility, and periodontal abscess. Radiographic examination revealed generalized horizontal bone loss. Lateral cephalogram revealed retarded somatic development. A diagnosis of PLS was arrived based on history, clinical and radiological findings. Nonsurgical periodontal treatment along with the treatment of skin lesions was performed. A combined and intensive mechanical and antimicrobial treatment with supportive periodontal treatment in PLS patients may halt the periodontal disease progression. The dentists should be aware of the same because an early diagnosis of the syndrome can help to preserve the teeth by the timely institution of treatment, using a multidisciplinary approach.

CLINICAL AND MOLECULAR ANALYSIS IN PAPILLON-LEFÈVRE SYNDROME

Papillon-Lefevre syndrome (PLS) is a cathepsin C (CTSC)-associated disorder characterized by palmar and plantar hyperkeratosis and aggressively progressing periodontitis. Here we report the clinical and genetic features of 5 PLS patients presenting with a severe periodontal breakdown in primary and permanent dentition, hyperkeratosis over the palms and soles, and recurrent sinusitis and/or tonsillitis. Mutation analysis revealed 2 novel homozygous recessive mutations (c.947 T>C and c.1010 G>C) and 1 previously described homozygous recessive mutation (c.901 G>A), with the parents heterozygous carriers, in 3 families (4 patients). The fourth family presented with the CTSC c.628 C>T heterozygous mutation, which was inherited maternally. The patient carrying the CTSC c.628 C>T mutation featured the classical PLS phenotype, but no PLS clinical characteristics were found in his carrier mother. These findings indicate that both homozygous and heterozygous mutations in the cathepsin C heavy chain domain may lead to the classical PLS phenotype, suggesting roles for epistasis or gene-environment interactions on determination of PLS phenotypes.

Oral rehabilitation of Papillon-Lefèvre syndrome patients by dental implants: a systematic review.

ObjectivesPapillon–Lefèvre syndrome (PLS) is a rare autosomal recessive disorder. These patients lose their teeth at a young age and are in need of prosthetic rehabilitation. The aim of this systematic review was to assess the success of dental implant placement in these patients.Materials and MethodsAn electronic search was performed in PubMed Central, Scopus, and Web of Science using the keyword “Papillon–Lefèvre syndrome” AND “dental implant” OR “prosthodontics”. Articles reporting implant placement in patients with PLS until July 2019 were included.ResultsAssessment of the included 11 articles reporting 15 cases showed 136 implant placements in these patients. Implant failure occurred in 3 patients (20 implants). The peri-implantitis and failure rate was higher in the maxilla. Meta-analysis showed the probability of failure to be 7% (95% confidence interval [CI] 0%-31%) for maxillary implants and 2% (95% CI 0%-9%) for mandibular implants. The follow-up time ranged between 1 and 20 years. Healing after bone graft and implant placement in these patients was uneventful.ConclusionDental implants may be a viable treatment option for PLS patients. Implantation can help preserve alveolar bone if the patients’ immunological and growing conditions are well-considered and proper oral hygiene and compliance with the maintenance program are continued.

Papillon-Lefèvre Syndrome: Detrimental Periodontal Condition in the Affected Individuals

This paper aims to highlight the detrimental periodontal condition in patients with Papillon-Lefèvre syndrome (PLS) and the pivotal role of a Periodontist in the diagnosis of the condition. PLS is also known as palmo-plantar keratosis (PPK) with an unusual periodontal status. Uncontrolled early-onset periodontitis, which affects both primary and permanent dentitions alike, is a hallmark of the syndrome. PLS patients’ exhibit defected neutrophil chemotactic function due to substandard activity of Cathepsin C (CTSC) gene. The result is the failure of elimination of periodontal pathogens that leads to severe periodontal destruction. We reported a case of an 11 years old Pakistani girl affected with PLS whose parents are consanguineously married. Since PLS is a rare autosomal recessive disorder and multiple consanguinity in a family, increases the risk of the occurrence of the syndrome in the off springs, the case report also highlights the importance of pre-marital genetic mapping and conception counseling for the families.

Papillon-Lefèvre syndrome: Oral aspects and treatment.

Papillon-Lefèvre syndrome (PLS) is a rare disorder characterized by diffuse palmoplantar erythematous, fissured hyperkeratosis, and aggressive periodontal disease that starts in the early periods of childhood. Periodontal disease occurs with the early loss of deciduous teeth at the age of 2 to 4 years, followed by the loss of permanent teeth during adolescence. Prosthodontics management of PLS patients is very complex and sometimes requires invasive therapeutic treatments. Early diagnosis is essential for correct treatment management avoiding the possibility that patients are early edentulous. Management could be a conventional periodontal treatment and pharmacological therapy but in severe cases, digital techniques, could be help the clinician for increased patient comfort and minimized tissue damage.

A Case Report: Papillon Lefevre Syndrome

Study Background: Papillon Lefevre Syndrome (PLS) is an autosomal recessive inherited genetic disorder characterized by palmoplantar hyperkeratosis and premature loss of deciduous and permanent teeth. Mutation of cathepsin C gene has been detected in the patient with PLS. In this case report, oral and radioghaphic findings of one PLS patient were discussed. 14 year old male patient diagnosed with PLS after radiographic, dermatologic and dental examination was referred to our clinic due to severe gingival inflamation and mobility. Severe gingival inflamation, alveolar bone resorption and multiple teeth loss due to periodontal reasons were observed. Mild palmar and moderate plantar hyperkeratosis were observed but no other systemic problems were detected. Methods: Non-surgical periodontal treatment was perfomed among multiple sessions and in each session subgingival tissues had been irrigated with %2 chlorhexidine irrigation solution. To reduce severe gingival inflammation antibiotherary has been prescribed. Hopeless teeth were extracted. Results: After non-surgical periodontal treatment and antibiotherary severe gingival inflammation was reduced but not eliminated completely, mobility score of some teeth reached to zero score, patient was motivated about oral hygiene and was taken into maintanence phases. After having extracted hopeless teeth patient was directed for prosthodontic rehabilitation. Conclusion: PLS is a rare autosomal recessive disorder. With PLS-specific dermatologic findings and characteristic periodontal view, it is possible to be able to identify the disease in its very early stages. Early diagnosis of the disease and institution of an appropriate periodontal and antimicrobial treatment might improve the prognosis.

Evaluation of Proteinase 3 Expression on Membrane of Papillon-Lefèvre Syndrome Neutrophils

Background: Proteinase 3, in its membrane form, is the main target antigen of anti-neutrophil autoantibodies in granulomatosis with polyangiitis (autoimmune disease). This neutrophil serine protease is synthesized as inactive zymogens at the early stage of neutrophil maturation and is activated by cathepsin C, the physiological activator of serine proteases. In neutrophils of Papillon-Lefevre syndrome patients, a genetic form of cathepsin C deficiency, amounts of intracellular proteinase 3 detected are very low or even undetectable. The aim of our study was to evaluate membrane expression of proteinase 3 on cathepsin C-deficient neutrophils by using the Papillon-Lefevre syndrome model in view of a therapeutic approach of granulomatosis with polyangiitis. Methods: We evaluated membrane expression of proteinase 3 on activated neutrophils of Papillon-Lefevre syndrome patients by cytometry. Results: Proteinase 3 was detected at neutrophils surface of Papillon-Lefevre syndrome patients, but significantly less than at healthy neutrophils surface. Conclusion: Pharmacological inhibition of cathepsin C may be an attractive therapeutic approach to eliminate the target autoantigen proteinase 3 of granulomatosis with polyangiitis patients.

Full-Mouth Rehabilitation with Calvarium Bone Grafts and Dental Implants for a Papillon-Lefèvre Syndrome Patient: Case Report

Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder of keratinization associated with palmoplantar keratoderma and severe periodontitis resulting in complete edentulism in late adolescence. The pathognomonic dental features of PLS are pathologic migration, hypermobility, and exfoliation of the teeth without any signs of root resorption. It has been suggested that an effective way to treat PLS patients presenting early in the disease progression is extraction of the erupted primary dentition or hopeless permanent teeth followed by antibiotic coverage with periodontal therapy for the remaining teeth. Unfortunately, studies have shown that this regimen only temporarily delays the progression of periodontal disease and does not prevent further tooth loss and bone destruction in the long term. Post-tooth loss, atrophic ridges make conventional prosthodontic rehabilitation quite challenging, and more recently, implant-supported prostheses have been considered as a viable alternative. In a PLS patient, implant placement is complicated by inadequate bone volume; thus, bone augmentation techniques or the use of short implants is often considered. When large volumes of bone are required, parietal calvarium bone can be used to predictably reconstruct severe defects. A PLS patient aged 21 years presented a chief complaint of ill-fitting conventional complete dentures. The patient had severely atrophic ridges, requiring significant bone augmentation for an implant-supported prosthesis. The present case is the first example of bone augmentation using autogenous calvarium parietal graft followed by endosseous implant placement and prosthetic restoration in a PLS patient.

Prolonged pharmacological inhibition of cathepsin C results in elimination of neutrophil serine proteases

Cathepsin C (CatC) is a tetrameric cysteine dipeptidyl aminopeptidase that plays a key role in activation of pro-inflammatory serine protease zymogens by removal of a N-terminal pro-dipeptide sequence. Loss of function mutations in the CatC gene is associated with lack of immune cell serine protease activities and cause Papillon-Lefèvre syndrome (PLS). Also, only very low levels of elastase-like protease zymogens are detected by proteome analysis of neutrophils from PLS patients. Thus, CatC inhibitors represent new alternatives for the treatment of neutrophil protease-driven inflammatory or autoimmune diseases. We aimed to experimentally inactivate and lower neutrophil elastase-like proteases by pharmacological blocking of CatC-dependent maturation in cell-based assays and in vivo. Isolated, immature bone marrow cells from healthy donors pulse-chased in the presence of a new cell permeable cyclopropyl nitrile CatC inhibitor almost totally lack elastase. We confirmed the elimination of neutrophil elastase-like proteases by prolonged inhibition of CatC in a non-human primate. We also showed that neutrophils lacking elastase-like protease activities were still recruited to inflammatory sites. These preclinical results demonstrate that the disappearance of neutrophil elastase-like proteases as observed in PLS patients can be achieved by pharmacological inhibition of bone marrow CatC. Such a transitory inhibition of CatC might thus help to rebalance the protease load during chronic inflammatory diseases, which opens new perspectives for therapeutic applications in humans.

Analysis of urinary cathepsin C for diagnosing Papillon-Lefèvre syndrome.

Papillon-Lefèvre syndrome (PLS) (OMIM: 245000) is a rare disease characterized by severe periodontitis and palmoplantar keratoderma. It is caused by mutations in both alleles of the cathepsin C (CatC) gene CTSC that completely abrogate the proteolytic activity of this cysteine proteinase. Most often, a genetic analysis to enable early and rapid diagnosis of PLS is unaffordable or unavailable. In this study, we tested the hypothesis that active CatC is constitutively excreted and can be easily traced in the urine of normal subjects. If this is true, determining its absence in the urine of patients would be an early, simple, reliable, low-cost and easy diagnostic technique. All 75 urine samples from healthy control subjects (aged 3 months to 80 years) contained proteolytically active CatC and its proform, as revealed by kinetic analysis and immunochemical detection. Of the urine samples of 31 patients with a PLS phenotype, 29 contained neither proteolytically active CatC nor the CatC antigen, so that the PLS diagnosis was confirmed. CatC was detected in the urine of the other two patients, and genetic analysis revealed no loss-of-function mutation in CTSC, indicating that they suffer from a PLS-like condition but not from PLS. Screening for the absence of urinary CatC activity soon after birth and early treatment before the onset of PLS manifestations will help to prevent aggressive periodontitis and loss of many teeth, and should considerably improve the quality of life of PLS patients.

Long-term change of disease behavior in Papillon-Lefèvre syndrome: Seven years follow-up

Papillon-Lefèvre syndrome (PLS) is an autosomal recessive disease, characterized by severe periodontitis and palmoplantar hyperkeratosis. Mutations in the cathepsin C (CTSC) gene are the causative genetic factor. PLS starts at very early age, however, the age associated change of PLS has never been characterized. In this report, four PLS patients with CTSC mutations were followed up for seven years, periodontal condition and serum immunoglobulins (Igs) were recorded. Results showed that periodontal inflammation of PLS peaked at teenage years, but declined with time. At the same time the serum IgE change was consistent with the change, suggesting the possibility of using IgE as a monitoring index for PLS inflammation level, or to develop new target for therapy.

Lack of cathelicidin processing in Papillon-Lefèvre syndrome patients reveals essential role of LL-37 in periodontal homeostasis.

BackgroundLoss-of-function point mutations in the cathepsin C gene are the underlying genetic event in patients with Papillon-Lefèvre syndrome (PLS). PLS neutrophils lack serine protease activity essential for cathelicidin LL-37 generation from hCAP18 precursor.AimWe hypothesized that a local deficiency of LL-37 in the infected periodontium is mainly responsible for one of the clinical hallmark of PLS: severe periodontitis already in early childhood.MethodsTo confirm this effect, we compared the level of neutrophil-derived enzymes and antimicrobial peptides in gingival crevicular fluid (GCF) and saliva from PLS, aggressive and chronic periodontitis patients.ResultsAlthough neutrophil numbers in GCF were present at the same level in all periodontitis groups, LL-37 was totally absent in GCF from PLS patients despite the large amounts of its precursor, hCAP18. The absence of LL-37 in PLS patients coincided with the deficiency of both cathepsin C and protease 3 activities. The presence of other neutrophilic anti-microbial peptides in GCF from PLS patients, such as alpha-defensins, were comparable to that found in chronic periodontitis. In PLS microbial analysis revealed a high prevalence of Aggregatibacter actinomycetemcomitans infection. Most strains were susceptible to killing by LL-37.ConclusionsCollectively, these findings imply that the lack of protease 3 activation by dysfunctional cathepsin C in PLS patients leads to the deficit of antimicrobial and immunomodulatory functions of LL-37 in the gingiva, allowing for infection with A. actinomycetemcomitans and the development of severe periodontal disease.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-014-0148-y) contains supplementary material, which is available to authorized users.

Long‐term results after treatment of periodontitis in patients with Papillon–Lefèvre syndrome: success and failure

Retrospective evaluation of periodontal status in patients with Papillon-Lefèvre syndrome (PLS) observed for ≥10years; identification of factors that may influence treatment outcome; and reporting of the outcome of dental implants in four PLS patients. All PLS patients currently registered at the Department of Periodontology, Goethe-University Frankfurt with a follow-up ≥10years (13-33years; mean 22years) were recruited. Eight patients (aged 17-46years) from five families (three pairs of siblings) were included. After comprehensive periodontal therapy in eight PLS patients, teeth were retained in only two. In six patients, all teeth were extracted, almost entirely due to periodontal reasons. In four patients, teeth were prosthodontically restored with implants. Currently, three patients already show peri-implantitis. In some PLS patients, periodontitis may be arrested by: combined mechanical and antibiotic periodontal treatment; extraction of severely diseased teeth; oral hygiene instructions; intensive maintenance therapy; and microbiological monitoring and treatment of the infection with Aggregatibacter actinomycetemcomitans. Implants in PLS patients who did not follow any maintenance programme have a high risk of peri-implantitis and implant loss. Treatment of PLS patients has always to be considered as high-risk cases.