Premature loss of primary teeth can occur as a consequence of dental trauma, neonatal tooth extraction, early childhood caries, or periodontal problems, or it can be a manifestation of systemic disease. This review aims to present systemic disorders that can lead to premature loss of deciduous teeth in children and to provide a comprehensive resource for clinical practice for both physicians and dentists.
This study is a narrative review of original studies and case reports published in English and Polish between 1957 and 2021 that was conducted by searching electronic scientific resources: PubMed, Google Scholar, Web of Science, and Science Direct. The schema of the qualification process is represented by a Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). In total, 196 articles were identified; after provisional assessment of the titles and abstracts by two reviewers, 46 were found to be relevant to the topic, including 1 review, 16 original papers, and 27 case reports regarding systemic disease resulting in premature tooth loss.
In this study, 16 systemic diseases were linked to premature primary tooth loss in children: Papillon-Lefèvre syndrome, mucocutaneous dyskeratosis, Coffin-Lowry syndrome, congenital adrenal hyperplasia, Langerhans cell histiocytosis, cherubism, hypophosphatasia, acatalasia, Chediak-Higashi syndrome, cyclic neutropenia, erythromelalgia, Down syndrome, Hajdu-Cheney syndrome, short bowel syndrome, leukocyte adhesion deficiency type 1 (LAD-1), and Wiedemann-Steiner syndrome (WSS).

Inherited phagocyte defects are one of the subgroups of primary immunodeficiency diseases (PIDs) with various clinical manifestations. As oral manifestations are common at the early ages, oral practitioners can have a special role in the early diagnosis.
A comprehensive search was conducted in this systematic review study and data of included studies were categorized into four subgroups of phagocyte defects, including congenital neutropenia, defects of motility, defects of respiratory burst, and other non-lymphoid defects.
Among all phagocyte defects, 12 disorders had reported data for oral manifestations in published articles. A total of 987 cases were included in this study. Periodontitis is one of the most common oral manifestations.
There is a need to organize better collaboration between medical doctors and dentists to diagnose and treat patients with phagocyte defects. Regular dental visits and professional oral health care are recommended from the time of the first primary teeth eruption in newborns.

OBJECTIVE: 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).
METHODS: 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.
RESULTS: 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%.
CONCLUSIONS: 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.
CONCLUSIONS: 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.

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Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder that exhibits palmoplantar keratosis and early severe periodontitis. The oral disease affects both the primary and permanent dentitions leading to premature exfoliation of teeth. Various etiologic factors, such as genetic mutations, immunologic alterations, and bacteria have been implicated in PLS. Genetic mutations leading to the loss of function of cathepsin C (CTSC) gene, located on chromosome 11q14, is considered pivotal in this condition. The present case series describes PLS in three siblings, with consanguineously married parents, who live in a remote area of Yemen. The affected children presented with prominent palmoplantar keratosis and early periodontitis with only a few remaining teeth. The severity of skin lesions in all patients exhibited seasonal variations. Based on their clinical findings, a diagnosis of PLS was made. Dentists have a significant role in the early diagnosis and management of PLS patients.

Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive condition that causes palmoplantar keratoderma and premature exfoliation of primary and secondary dentition. There are several diseases that can mimic PLS, and it is estimated that the disease is far more prevalent than documented but may either be misdiagnosed or not acknowledged. Radiological exams can provide important clues to differentiate between these syndromes and make an accurate diagnosis.

Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, is a rare, proliferative disorder in which the accumulation of pathologic Langerhans cells leads to local tissue infiltration and destruction. The incidence of LCH is estimated to be one case per 200,000 children per year. The role of the dentist is important in early and accurate evaluation, staging and diagnosis of LCH, because it may mimic more common diseases, such as juvenile periodontitis and osteomyelitis. There are multiple treatment options, but the response is unpredictable. The aim of this paper is to give a short, introductory overview on current diagnostic and treatment strategies for LCH in the oral and maxillofacial region and to present a case of LCH that mimicked juvenile periodontitis and was resolved following extraction of affected teeth. The history, radiological appearance, differential diagnosis, histopathology and treatment options for the patient are discussed.

The Papillon-Lefevre syndrome (PLS) was first described by Papillon and Lefevre in 1924. It is an autosomal recessive disorder characterized by a diffuse palmoplanter hyperkeratosis and rapidly progressive and devastating periodontitis, affecting the primary as well as the permanent dentition, attributed to a point mutation of the cathepsin C gene. This paper presents a clinical presentation and a brief review of its etiology and treatment modalities.

暂无摘要。

Various approaches for treating the periodontal condition associated with Papillon-Lefèvre syndrome (PLS) have been reported. These include oral hygiene instruction, use of mouth rinses, frequent debridement, multiple antibiotic regimens, periodontal surgery, extraction of hopeless teeth, and extraction of all primary teeth. The objective of this paper is to review the literature on the different treatment approaches of patients with PLS and to illustrate the importance of early diagnosis as well as treatment in long-term maintenance of oral health. Because PLS is rare, most publications are case reports, and very few document long-term successful treatment of the periodontal condition. Several treatment approaches are summarized in this paper with the long-term reports. The importance of early diagnosis is highlighted. The recommendation is that young patients with skin lesions and severe periodontal destruction together with premature loss of teeth should be referred to a periodontist as early as possible. This should prevent or delay tooth loss and enhance early replacement of missing teeth for preservation of function and esthetics. In conclusion, successful periodontal management of PLS patients remains challenging. However, as our understanding of the etiological factors increases, it is hoped that successful treatment strategies will be developed.