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

Nagaveni, N B; Suma, R; Shashikiran, N D; Reddy, V V Subba

doi:10.4103/0970-4388.41622

Cited by 22 publications

(35 citation statements)

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“…Chemotactic and phagocytic function of neutrophils is decreased in PLS, and it is associated with a diminished phytohemagglutinin response by T-cell lymphocytes [9, 12]. In consistence with previous reports, consanguineous marriage was found in fifty percent of our patients implying the genetic basis for the disease [14, 15]. Due to low socioeconomic status, genetic testing was not performed to identify responsible mutations.…”

Section: Discussionsupporting

confidence: 64%

Papillon-Lefèvre Syndrome: A Series of Six Cases in the Same Family

et al. 2012

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Papillon-Lefèvre syndrome (PLS) is a rare, autosomal recessive heterogeneous disorder, which is characterized by palmoplantar hyperkeratosis, early loss of primary and permanent teeth, and associated calcification of the dura mater. Herein we described six cases of PLS in the same family. In this series, six cases (two females and four males) with the mean age of 15.6 ± 10.4 years were recruited. Palmoplantar hyperkeratosis was detected in all of the cases, leading to a difficult and painful walking in two cases due to lesions on the soles. Skin lesions were sharply distinct from adjacent normal skin in all cases. Other skin lesions were located in the external malleolus (5/6), knee (4/6), elbow (4/6), toe and dorsal fingers (3/6), and the thighs (2/6). In three cases, all permanent teeth were exfoliated. In three others, no primary teeth remained. Severe gingivitis was observed in three patients. Radiologic study confirmed alveolar bone destruction in five cases. Delayed diagnosis and insufficient treatment of PLS patients can affect patient's life of by causing edentulism at a young age and may impose PLS patients to increased risk of social, psychological, and economical burdens.

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Section: Discussionsupporting

confidence: 64%

Papillon-Lefèvre Syndrome: A Series of Six Cases in the Same Family

et al. 2012

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“…[1] Resorption of the alveolar bone gives the teeth a “floating-in-air” appearance on dental radiographs. [5]…”

Section: Introductionmentioning

confidence: 99%

Papillon-lefevre syndrome: A combined approach from the dermatologist and dentist - A clinical presentation

et al. 2011

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Papillon–Lefevre syndrome (PLS) is a rare disease characterized by skin lesions, which includes palmar-plantar hyperkeratosis and hyperhidrosis with severe periodontal destruction involving both the primary and the permanent dentitions. It is transmitted as an autosomal-recessive condition, and consanguinity of parents is evident in about one-third of the cases. This paper describes a 13-year-old male patient who presented to the department of pedodontics, with rapidly progressing periodontitis. A general physical examination revealed scaling on the hands and feet, which had been medically diagnosed as PLS. The incidence of this rare entity is increasing in the recent times, which is associated with irreparable periodontal destruction at an early age, with not so prominent skin lesions in some cases. In such instances, the dentist has a more important role in diagnosing, treatment planning and preservation of the periodontal tissues and, at the same time, referring for the treatment of the skin lesions. This paper emphasizes the combined effort of the two specialities in order to maintain skin as well as dental conditions in health by early intervention and a synergistic treatment approach.

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“…The patient in this report had no structural deformities in her upper and lower extremities. [7][8][9][10][11][12][13] Psychosocial condition of a patient with PLS tends to be affected because of losing teeth at a young ages. Therefore, early dental evaluation is a significant step in psychological rehabilitation for children with PLS.…”

Section: Discussionmentioning

confidence: 99%

“…3 Microbiological studies of plaque samples from patients with PLS have shown Actinobacillus actinomycetemcomitans, Porphyromonas gingivalis, Fusobacterium nucleatum, and Treponema denticola organisms as examples of many pathogens that may be involved in this disease. 7 A. actinomycetemcomitans is most responsible in the pathogenesis of the rapid periodontal destruction in patients with PLS. 8 In addition to the dermatologic and oral findings, patients may have decreased neutrophil, lymphocyte, or monocyte functions and an increased susceptibility to bacterial infection, leading to persistent pyogenic infections of the skin.…”

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confidence: 99%

Treatment of Patient With Papillon-Lefevre Syndrome With Short Dental Implants: A Case Report

Etöz¹,

Ulu²,

Kesim³

2010

Implant Dentistry

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Papillon-Lefevre syndrome (PLS) is an autosomal recessive disorder. Main features of PLS are hyperkeratosis of palms and soles concomitant with premature loss of primary and permanent dentition due to progressive periodontitis. Dental management of patients with PLS is usually challenging because of early excessive loss of alveolar bone support. We describe dental rehabilitation of a 34-year-old patient with PLS with severely atrophic mandible by means of 2 short (6 mm) dental implants between 2 mental foramina supporting an implant retained complete denture. After 1 year of follow-up period, the patient was doing well and there was no sign of bone resorption. In patients with PLS, dental osseointegrated implants (even with shorter lengths) can be safely used for atrophic mandibles instead of invasive preprosthetic applications such as bone augmentation, nerve lateralization, or alveolar distraction to avoid possible complications.

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

Cited by 22 publications

References 0 publications

Papillon-Lefèvre Syndrome: A Series of Six Cases in the Same Family

Papillon-Lefèvre Syndrome: A Series of Six Cases in the Same Family

Papillon-lefevre syndrome: A combined approach from the dermatologist and dentist - A clinical presentation

Treatment of Patient With Papillon-Lefevre Syndrome With Short Dental Implants: A Case Report

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