Case Of Papillon-Lefevre Syndrome Research Articles

An observational study of various oral manifestations in 100 patients visiting a tertiary care hospital

Context: The oral lesions may be the earliest manifestation of the dermatological diseases or the most significant clinical appearance of some dermatological diseases and occasionally lesions occur simultaneously in the skin as well as mucous membrane.Aims: To study the clinical patterns and frequency of oral lesions.Settings and Design: A descriptive observational study.Methods and Material: It was a hospital based observational study, that was conducted in thedepartment of dermatology of a tertiary care hospital over a period of 24 months. A total number of 100 individuals with oral lesions were included in the study. A detailed history was taken and complete clinical examination was done. Histological and other investigations were done only if essential to establish the diagnosis.Results: In this study, we studied oral lesions in 100 patients, showing female preponderance 53%cases. Most of the patients belonged to 3rd and 4th decade of life 20% and 26% respectively. Among 37 cases of recurrent oral ulcers seen in the study comprised 37.83% cases each of pemphigus group and herpetic stomatitis. The remaining 24.32% were of recurrent aphthae. The 30 cases of oral ulcers seen in the study comprised 33.3% cases, aphthous 30%, neoplastic 23.3%, local trauma 13.3%. The 9 cases of drug induced oral ulcers seen in this study comprised 22.2% each of drug induced cheilitis, SJS, TEN, perioral dermatitis and 11.1% cases of bullous FDE. The 37 cases of oral lesions seen in the study comprised vesiculobullous disorders in 35.13%, reaction pattern in 24.3%, lichen planus in 24.3%, 5.4% each of TEN, SJS, EM and 2.7% of bullous FDE. The 19 cases of infectious etiology leading to oral ulcers comprised of viral infection in 78.9% and fungal infection in 21.1%. The 12 cases of neoplasms comprised of benign in 41.6% cases, premalignant in 33.3% cases, and malignant in 25% of cases. In this study, genodermatosis cases were six in our study cases, four of which were of ectodermal dysplasia, one case of Papillon Lefevre syndrome, one case of neurofibromatosis-1. In this study, most common 58% involved sites were the periodontium, gingivae and buccal mucosa combined. Involvement of the lips 22.2% was the next significant site. Tongue, teeth, salivary glands, palate were 8%, 5%, 4%, 3% respectively.Conclusions: This study brings into focus prevalence of various oral manifestations. Thorough knowledge about oral manifestations is essential to arrive at a specific diagnosis which is a mandate for counselling and management.

掌跖角化-牙周破坏综合征合并先天性眼病一例

掌跖角化-牙周破坏综合征合并先天性眼病一例

Report of a case of Papillon-Lefevre syndrome

Papillon-Lefevre Syndrome is a rare, autosomal recessive disorder. It is characterized by dermatological and periodontal changes that include palmar-plantar hyperkeratosis and generalized aggressive periodontitis in both primary and permanent dentition. Hiam-Munk syndrome and hypophosphatasia are some of its differential diagnoses. Dentists and dermatologists both should be aware of its characteristics which help to have an early diagnosis, a multidisciplinary approach and better prognosis. Here, we present a case of Papillon-Lefevre Syndrome in a 4-year-old girl who was brought to a private dental office with the chief complaint of tooth mobility.

Genetic Mapping in Papillon-Lefèvre Syndrome: A Report of Two Cases

Papillon-Lefevre syndrome (PLS) is a rare autosomal recessive heterogeneous trait which is characterized by erythematous palmoplantar hyperkeratosis, early-onset periodontitis, and associated calcification of dura mater. The etiology of PLS is multifactorial with genetic, immunological, and microbial factors playing a role in etiopathogenesis. Recently identified genetic defect in PLS has been mapped to chromosome 11q14–q21, which involves mutations of cathepsin C. This paper presents a report of 2 cases of Papillon-lefevre syndrome in which diagnosis is based on clinical presentation and genetic mapping.

A Case Report of Papillon - Lefevre Syndrome

Papillon-Lefevre syndrome is a rare autosomal recessive genetic disorder in which there is a palmoplanter keratinisation and premature loss of both deciduous and permanent teeth. Consanguinity of parents is evident in about one third of cases. Earlv diagnosis of the syndrome can help to preserve the teeth by early institution of treatment, using a multidisciplinary approach. Here we describe a case of Papillon-Lefevre syndrome along with the clinical features, differential diagnosis and management of this syndrome are discussed.

Papillon Lefevre syndrome: A periodontist approach

Introduction: Papillon-Lefevre Syndrome (PLS) is a rare autosomal recessive disorder of keratinisation, characterized by palmoplantar hyperkeratosis, periodontitis and early loss of dentition. Since these features are common to both dentistry and dermatology, members of both disciplines should be aware of the same because an early diagnosis of this condition can help to preserve the teeth by early institution of treatment, using a multidisciplinary approach. Case Series: The authors here present two cases of Papillon-Lefevre syndrome in siblings, having all of the characteristic features, along with a comprehensive review of the etiology, pathology, clinical features, differential diagnosis and management of the condition. Conclusion: The main priority of physician's strategy is to refer the PLS patients at the earliest to a periodontist for the periodontal management of permanent dentition. Based on the previously reported series and our clinical experience, non-surgical periodontal therapy yielded better results when started immediately after the extraction of deciduous dentition.

Papillon-Lefevre syndrome: Report of two cases in the same family

Papillon-Lefevre syndrome is a very rare syndrome of autosomal recessive inheritance characterized by palmar-plantar hyperkeratosis and early onset of a severe destructive periodontitis, leading to premature loss of both primary and permanent dentitions. Various etiopathogenic factors are associated with the syndrome but a recent report has suggested that the condition is linked to mutations of the cathepsin C gene. Two cases of Papillon-Lefevre syndrome in the same family, having all of the characteristic features, are presented. An 11-year-old girl and a 9-year-old boy presented with the complaints of loose teeth. Both expressed hyperkeratosis of palms, soles, and knees. Severe generalized periodontal destruction, with mobility of teeth, was evident on intraoral examination; orthopantomograph examination showed severe generalized loss of alveolar bone in both the patients.

Juvenile periodontitis (periodontosis) in a Nigerian teaching hospital.

A STUDY OF the pattern of juvenile periodontitis (periodontosis) as observed in the University College Hospital, Ibadan was carried out, through a period of one year. The age group was only confined from 14-25 years, and the patients selected for the study were only those patients in the above age group reporting to the Hospi tal's Dental Centre, immaterial of what the patients' main complaint was. It was revealed that there was a high rate of patients attending this Institution with juvenile periodontitis. Further it is quite alarming to note that all the patients examined on their first visits had a clinically observable and detactable chronic gingivitis, some even transitioned to a chronic periodontitis. Routine investigations further revealed a few cases of undetected diabetes and one case of Papillon-Lefevre Syndrome (hyperkeratosis palmoplantaris).

A Case of Papillon-Lefevre Syndrome and its Review of the Literature

A Case of Papillon-Lefevre Syndrome and its Review of the Literature

PAPILLON‐LEFEVRE SYNDROME

Two cases of Papillon-Lefevre Syndrome (PLS) in a family are reported. Association of recurrent infections, arachnodactyly, intracranial calcification and phalangeal abnormality have been observed. Because there is no history of consanguinity, and autosomal recessive pattern of inheritance is suggested.

Papillon-Lefevre syndrome: report of two cases in the same family

Two cases of Papillon-Lefevre syndrome have been seen in the same family. The characteristic features of the syndrome are shown to be present in both children and the main characteristics are hyperkeratosis of the palms of the hands and soles of the feet; destruction of the periodontium of the deciduous teeth with subsequent premature loss of the teeth; and destruction of the periodontium of the permanent teeth and the premature loss of these teeth usually before 16 years of age.