Papillon-Lefevre syndrome (PLS) is a rare, autosomal recessive inherited, type IV palmoplantar keratoderma (Hart, et al., 2000). Its prevalence is approximately 4 cases per one million people with no sex or racial predominance (Sreeramulu, et al., 2015). Approximately 1 to 4 people per thousand are carriers of the disorder (Sreeramulu, et al., 2015). If both parents are carriers, there is only a 25% chance that their offspring will be affected. If both parents are affected with PLS, there is a 100% chance that their offspring will be affected. Thus, PLS is frequent in consanguineous families and such relationships are seen in over 50% of all known cases of PLS (Sulak, et al., 2014). Papillon-LeFevre syndrome is caused by a mutation on chromosome
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Individuals with PLS are commonly afflicted with periodontal disease that affects both deciduous and succedaneous teeth, eventually leading to edentulism (Sulak, et al., 2014). In patients with PLS, deciduous teeth erupt at the average expected age and follow the normal sequence and shape of teeth as in a healthy individual (Sreeramulu, et al., 2015). In some cases, however, patients may exhibit microdontia or incomplete root formation (Sreeramulu, et al., 2015). Shortly after eruption, the gingiva becomes inflamed and periodontal structures deteriorate. Teeth become mobile, making mastication uncomfortable or painful, pus exudes out of periodontal abscesses, and fetid mouth odor is usually present (Sreeramulu, et al., 2015). The deciduous teeth are lost prematurely at around the age of 4 or 5 and the gingiva returns to its normal state (Sreeramulu, et al., 2015). However, as the permanent teeth begin to erupt, the vicious cycle repeats all over again: the gingiva becomes inflamed and the teeth exhibit hypermobility, drifting, and exfoliation (Sreeramulu, et al., 2015). The permanent teeth are prematurely shed and the patient is edentulous as early as his or her teen years. Radiographs of patients with PLS also often show vertical alveolar bone loss around the incisors and first molars, distinguishing PLS from chronic periodontitis in which horizontal alveolar bone …show more content…
However, most laboratory tests of patients with PLS return with results within normal limits (Sreeramulu, et al., 2015). The results are useful only in that it eliminates the possibility of other disorders that could have caused gingival inflammation and premature loss of teeth. Of particular help are the neutrophil-function test and intraoral radiographs. A neutrophil-function test of a patient with PLS will show reduced response to certain bacteria, such as Staphylococcus spp. and A. actinomycetemcomitans (Sreeramulu, et al., 2015). An intraoral radiograph of a patient with PLS will show vertical bone loss around the incisors and first molars and teeth that appear to be floating in air (Sreeramulu, et al., 2015). The real challenge, however, is not the diagnosis of PLS; rather, it is the creation of a treatment plan that will effectively mitigate the symptoms of PLS. Early diagnosis and intervention can delay loss of teeth. Much of the treatment, however, still remains a challenge. Health professionals hope that as modern science continues to unravel the etiology and pathogenesis behind PLS, they will be able to develop more effective treatment strategies for their
Individuals with PLS are commonly afflicted with periodontal disease that affects both deciduous and succedaneous teeth, eventually leading to edentulism (Sulak, et al., 2014). In patients with PLS, deciduous teeth erupt at the average expected age and follow the normal sequence and shape of teeth as in a healthy individual (Sreeramulu, et al., 2015). In some cases, however, patients may exhibit microdontia or incomplete root formation (Sreeramulu, et al., 2015). Shortly after eruption, the gingiva becomes inflamed and periodontal structures deteriorate. Teeth become mobile, making mastication uncomfortable or painful, pus exudes out of periodontal abscesses, and fetid mouth odor is usually present (Sreeramulu, et al., 2015). The deciduous teeth are lost prematurely at around the age of 4 or 5 and the gingiva returns to its normal state (Sreeramulu, et al., 2015). However, as the permanent teeth begin to erupt, the vicious cycle repeats all over again: the gingiva becomes inflamed and the teeth exhibit hypermobility, drifting, and exfoliation (Sreeramulu, et al., 2015). The permanent teeth are prematurely shed and the patient is edentulous as early as his or her teen years. Radiographs of patients with PLS also often show vertical alveolar bone loss around the incisors and first molars, distinguishing PLS from chronic periodontitis in which horizontal alveolar bone …show more content…
However, most laboratory tests of patients with PLS return with results within normal limits (Sreeramulu, et al., 2015). The results are useful only in that it eliminates the possibility of other disorders that could have caused gingival inflammation and premature loss of teeth. Of particular help are the neutrophil-function test and intraoral radiographs. A neutrophil-function test of a patient with PLS will show reduced response to certain bacteria, such as Staphylococcus spp. and A. actinomycetemcomitans (Sreeramulu, et al., 2015). An intraoral radiograph of a patient with PLS will show vertical bone loss around the incisors and first molars and teeth that appear to be floating in air (Sreeramulu, et al., 2015). The real challenge, however, is not the diagnosis of PLS; rather, it is the creation of a treatment plan that will effectively mitigate the symptoms of PLS. Early diagnosis and intervention can delay loss of teeth. Much of the treatment, however, still remains a challenge. Health professionals hope that as modern science continues to unravel the etiology and pathogenesis behind PLS, they will be able to develop more effective treatment strategies for their