scholarly journals Primary Oro-Facial Manifestations of Langerhans Cell Histiocytosis in Pediatric Age: A Bi-Institutional Retrospective Study on 45 Cases

Children ◽  
2020 ◽  
Vol 7 (9) ◽  
pp. 104
Author(s):  
Saverio Capodiferro ◽  
Angela Tempesta ◽  
Luisa Limongelli ◽  
Giuseppe Ingravallo ◽  
Eugenio Maiorano ◽  
...  
Keyword(s):  
Bone Loss ◽  
Head And Neck ◽  
Langerhans Cell Histiocytosis ◽  
Alveolar Bone ◽  
Early Stage ◽  
Neck Region ◽  
Pediatric Dentistry ◽  
Langerhans Cell ◽  
Clinical Appearance ◽  
Periodontal Tissues

Aims: Langerhans Cell Histiocytosis is a rare hematologic disorder usually affecting children and most commonly involving the head and neck region. Primary oro-facial manifestations are rare, and their diagnosis is often challenging as they are numerous and often resemble common pathologies, refractory to conventional medical and/or instrumental treatments. For such reasons, the diagnosis is frequently delayed, as is the following staging and therapy onset. We retrospectively studied 45 pediatric patients affected by Langerhans Cell Histiocytosis with onset in the head and neck, to examine their clinical and radiological features at the early stage. Materials and Methods: The study was a retrospective bi-institutional analysis (Department of Pediatric Dentistry and Pediatric Oncology of “Sapienza” University of Rome, Department of Interdisciplinary Medicine of the University of Bari “Aldo Moro”), which enrolled 45 patients (age range 0–18 year-old) affected by Langerhans Cell Histiocytosis with oro-facial onset. Data regarding clinical appearance, number, site, synchronous or metachronous occurrence, involved tissues/organs, radiographic features and clinical outcomes were collected, listed and overall differentiated by two age ranges (0–10-year-olds and 10–18-year-olds). Results: Patients were 26 males and 19 females, with an average age at the time of diagnosis of 4.8 ± 3.8 years (median = 3.9 years). The most common findings were inflamed, hyperplastic, painful and often ulcerated gingival lesions (22 cases), associated with deciduous tooth mobility and/or dislocation with bone loss in 18 cases, followed by nine single eosinophilic granulomas of the mandible and two of the maxilla. Lesions of the palatal mucosa were observed in six patients; nine patients showed on radiograms the characteristic “floating teeth” appearance in the mandible with synchronous lesions of the maxilla in six. Paresthesia was relatively un-frequent (three cases) and the pathological fracture of the mandible occurred in six. Head/neck lymph nodes involvement was associated with oral lesions in 12 cases and skull lesions in 14. Otitis (media or externa) was detected in four instances, exophthalmia in two, cutaneous rush in nine, contextual presence or subsequent onset of insipidus diabetes in eight. As for therapy, single or multiple small jaw lesions were all surgically removed; chemotherapy with vinblastine alone or associated with corticosteroids was the principal treatment in almost the 80% of cases; more than 50% of patients received corticosteroids, while only three patients received adjunctive radiotherapy. The overall mortality account for less than 9% (four of 45 cases) and recurrence observed in eight patients after therapy. Conclusions: Langerhans Cell Histiocytosis may mimic several oro-facial inflammatory and neoplastic diseases. Considering the potential disabling sequela following head and neck localization of Langerhans Cell Histiocytosis in children, especially at the periodontal tissues with teeth and alveolar bone loss, lesion recognition along with the histological examination of suspicious tissues is mandatory to achieve an early diagnosis and to prevent further organ involvement.

scholarly journals Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Hesam Jahandideh ◽  
Yasser Nasoori ◽  
Sara Rostami ◽  
Mahdi Safdarian
Keyword(s):  
Head And Neck ◽  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Neck Region ◽  
Rare Condition ◽  
Neck Mass ◽  
Langerhans Cell ◽  
Adult Woman ◽  
Head And Neck Region ◽  
Laryngeal Involvement

Langerhans cell histiocytosis (LCH) is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease.

High Prevalence of BRAF V600E Mutations in Langerhans Cell Histiocytosis of Head and Neck in Chinese Patients

2019 ◽  
Vol 27 (8) ◽  
pp. 836-843
Author(s):  
Xiaoxiao Liu ◽  
Ye Zhang ◽  
Chuan-Xiang Zhou
Keyword(s):  
Head And Neck ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Neck Region ◽  
Braf V600e Mutation ◽  
Langerhans Cell ◽  
Braf V600e ◽  
Chinese Patients ◽  
Clonal Proliferation ◽  
High Prevalence

Langerhans cell histiocytosis (LCH) is characterized by clonal proliferation of Langerhans cells and has been classified as a hematolymphoid tumor. BRAF V600E mutation was found to be frequent in LCH; however, it has also been reported that Asia patients with LCH tend to show a lower rate of BRAF V600E mutation. In this study, we found LCH from the head and neck region often involved bone especially the posterior of the mandible and presented a high prevalence of BRAF V600E mutation in Chinese patients. Our findings also showed immunohistochemical detection correlated very well to DNA sequencing of BRAF alterations, which may be useful in the diagnosis of LCH, especially in cases with a low proportion of Langerhans cells, and BRAF inhibitors might be a treatment option for patients with LCH harboring BRAF V600E mutation.

Head and Neck Langerhans Cell Histiocytosis

1997 ◽  
Vol 106 (6) ◽  
pp. 526-532 ◽  
Author(s):  
Kenneth O. Devaney ◽  
Alfio Ferlito ◽  
Mathew J. Putzi ◽  
Alessandra Rtnaldo
Keyword(s):  
Head And Neck ◽  
Langerhans Cell Histiocytosis ◽  
Neck Region ◽  
Langerhans Cell ◽  
Skeletal System ◽  
Head And Neck Region ◽  
The Past ◽  
System Disease ◽  
Secondary Infections ◽  
Multifocal Disease

Among the potential sites of involvement by Langerhans cell histiocytosis (LCH), the head and neck region is the most commonly cited. Though principally a pediatric disease, LCH can affect any age group. It can be unifocal (skeletal) or multifocal (skeletal and/or visceral); it appears as though the presence of visceral lesions is more common in the youngest patients, and may be associated in some with a rapidly progressive course resulting in death. Head and neck manifestations may mimic such varied entities as eczema, otitis media, osteomyelitis, and cholesteatoma. Current approaches to therapy are less aggressive than they were in the past, and are particularly intended to monitor for and treat any complicating secondary infections (which may develop in the youngest patients with multifocal disease including visceral involvement). The prognosis is very good for unifocal skeletal system disease, and poor for multifocal disease with involvement of tissues other than bone.

scholarly journals Mouse Model of Loeys–Dietz Syndrome Shows Elevated Susceptibility to Periodontitis via Alterations in Transforming Growth Factor-Beta Signaling

2021 ◽  
Vol 12 ◽  
Author(s):  
Satoru Yamada ◽  
Kenichiro Tsushima ◽  
Masaki Kinoshita ◽  
Hiromi Sakashita ◽  
Tetsuhiro Kajikawa ◽  
...  
Keyword(s):  
Growth Factor ◽  
Bone Loss ◽  
Mouse Model ◽  
Mrna Expression ◽  
Missense Mutation ◽  
Transforming Growth Factor ◽  
Alveolar Bone ◽  
Elastic Fibers ◽  
Periodontal Tissues ◽  
Significant Difference

Loeys–Dietz syndrome (LDS) is a syndromic connective tissue disorder caused by a heterozygous missense mutation in genes that encode transforming growth factor (TGF)-β receptor (TGFBR) 1 and 2. We encountered a patient with LDS, who had severe periodontal tissue destruction indicative of aggressive periodontitis. The patient had a missense mutation in the glycine and serine-rich domain of TGFBR1 exon 3. This G-to-T mutation at base 563 converted glycine to valine. We established an LDS model knock-in mouse that recapitulated the LDS phenotype. Homozygosity of the mutation caused embryonic lethality and heterozygous knock-in mice showed distorted and ruptured elastic fibers in the aorta at 24 weeks of age and died earlier than wildtype (WT) mice. We stimulated mouse embryonic fibroblasts (MEFs) from the knock-in mouse with TGF-β and examined their responses. The knock-in MEFs showed downregulated Serpine 1 mRNA expression and phosphorylation of Smad2 to TGF-β compared with WT MEFs. To clarify the influence of TGF-β signaling abnormalities on the pathogenesis or progression of periodontitis, we performed pathomolecular analysis of the knock-in mouse. There were no structural differences in periodontal tissues between WT and LDS model mice at 6 or 24 weeks of age. Micro-computed tomography revealed no significant difference in alveolar bone resorption between WT and knock-in mice at 6 or 24 weeks of age. However, TGF-β-related gene expression was increased significantly in periodontal tissues of the knock-in mouse compared with WT mice. Next, we assessed a mouse periodontitis model in which periodontal bone loss was induced by oral inoculation with the bacterial strain Porphyromonas gingivalis W83. After inoculation, we collected alveolar bone and carried out morphometric analysis. P. gingivalis-induced alveolar bone loss was significantly greater in LDS model mice than in WT mice. Peritoneal macrophages isolated from Tgfbr1G188V/+ mice showed upregulation of inflammatory cytokine mRNA expression induced by P. gingivalis lipopolysaccharide compared with WT macrophages. In this study, we established an LDS mouse model and demonstrated that LDS model mice had elevated susceptibility to P. gingivalis-induced periodontitis, probably through TGF-β signal dysfunction. This suggests that TGF-β signaling abnormalities accelerate the pathogenesis or progression of periodontitis.

scholarly journals Effects of cigarette smoking on periodontium

2002 ◽  
Vol 55 (5-6) ◽  
pp. 229-232 ◽  
Author(s):  
Marija Bokor-Bratic
Keyword(s):  
Bone Loss ◽  
Oral Hygiene ◽  
Dental Plaque ◽  
Alveolar Bone ◽  
Gingival Recession ◽  
Tobacco Consumption ◽  
Alveolar Bone Loss ◽  
Age Difference ◽  
Periodontal Tissues ◽  
Periodontal Condition

Introduction The exact mechanisms by which smoking effects the periodontal tissues are not known. Studies in which plaque or calculus are taken into consideration come to conflicting conclusions regarding effects of smoking. Aim The aim of this study was to examine the oral hygiene and periodontal status in smokers and compare them with nonsmokers. Material and methods The study group comprised 83 smokers and 83 nonsmokers. The mean age (SD) of smokers and nonsmokers was 42,4?7,0 years and 43,7?6,4 years, respectively. The age difference was not statistically significant. The average tobacco consumption of the smokers at the time of investigation was 14 cigarettes a day and they had been regular smokers for 21 years on average. Results The amount of dental plaque was evaluated in accordance with the criteria of Green-Vermillion by using disclosing solution. The periodontal condition was evaluated by Ramfjord Periodontal Disease Index. For gingival recession the distance from the cemento-enamel junction to the gingival margin was determined on mid-buccal and mid-lingual surfaces of all teeth. Each subject was radiographically examined with a full mouth intraoral survey. Alveolar bone loss was determined as the distance from the cemento-enamel junction to the point where lamina dura became continuous with the compact bone of the interdental septum. Mean alveolar bone loss based on all mesial and distal measurements was calculated for each subject. The amount of dental plaque was high in both smokers (2,60,60) and nonsmokers (1,50,70), whereas the differences were statistically significant (p<0.001). Conclusion Periodontal destruction, alveolar bone loss and gingival recession were significantly increased in smokers compared to nonsmokers (p<0.001). It is concluded that differences observed between smokers and nonsmokers with regard to periodontal condition are attributable to differences in oral hygiene. Smoking is a risk factor for periodontal health.

scholarly journals Revealing a rare inflammatory oral manifestation in a 6-year-old child

2019 ◽  
Vol 12 (7) ◽  
pp. e229483
Author(s):  
Samapika Routray ◽  
Amit Kumar Adhya ◽  
Joseph John ◽  
Punit Dikhit
Keyword(s):  
Bone Loss ◽  
Buccal Mucosa ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Oral Manifestation ◽  
Atypical Cells ◽  
History Of ◽  
Permanent Molars ◽  
Superficial Dermis ◽  
Distal Aspect

A 6-year-old child with an episodic history of ulcerations over buccal mucosa was found to have severe inflammation on the palatal aspect of permanent first molars with grade 2 mobility bilaterally. Radiographical features were suggestive of bone loss around permanent molars extending to the distal aspect of the deciduous first molars. The clinical and radiographical findings were indicative of periodontal degeneration without any apparent cause visible intraorally. Further biopsy was done from the rashes present on the malar prominences, which showed nodular aggregates of atypical cells in superficial dermis. These large histiocytic cells with vesicular nuclei and nuclear grooves were immunopositive for CD1a and S100, concluding the diagnosis of Langerhans cell histiocytosis. For treatment, patient was referred to Department of Haemato-oncology and chemotherapy was suggested as per protocol.

Involvement of Cot/Tp12 in Bone Loss during Periodontitis

2010 ◽  
Vol 89 (2) ◽  
pp. 192-197 ◽  
Author(s):  
T. Ohnishi ◽  
A. Okamoto ◽  
K. Kakimoto ◽  
K. Bandow ◽  
N. Chiba ◽  
...  
Keyword(s):  
Bone Loss ◽  
Alveolar Bone ◽  
Alveolar Bone Loss ◽  
Periodontal Tissue ◽  
Rankl Expression ◽  
Periodontal Tissues ◽  
Tnf Α ◽  
Experimental Periodontitis ◽  
Deficient Mice

Periodontitis causes resorption of alveolar bone, in which RANKL induces osteoclastogenesis. The binding of lipopolysaccharide to Toll-like receptors causes phosphorylation of Cot/Tp12 to activate the MAPK cascade. Previous in vitro studies showed that Cot/Tp12 was essential for the induction of RANKL expression by lipopolysaccharide. In this study, we examined whether Cot/Tp12 deficiency reduced the progression of alveolar bone loss and osteoclastogenesis during experimental periodontitis. We found that the extent of alveolar bone loss and osteoclastogenesis induced by ligature-induced periodontitis was decreased in Cot/Tp12-deficient mice. In addition, reduction of RANKL expression was observed in periodontal tissues of Cot/Tp12-deficient mice with experimental periodontitis. Furthermore, we found that Cot/Tp12 was involved in the induction of TNF-α mRNA expression in gingiva of mice with experimental periodontitis. Our observations suggested that Cot/Tp12 is essential for the progression of alveolar bone loss and osteoclastogenesis in periodontal tissue during experimental periodontitis mediated through increased RANKL expression.

scholarly journals Clinicopathological and Immunohistochemical Study of Head and Neck Langerhans Cell Histiocytosis from Latin America

2017 ◽  
Vol 12 (4) ◽  
pp. 431-439 ◽  
Author(s):  
Natália Rocha Bedran ◽  
Román Carlos ◽  
Bruno Augusto Benevenuto de Andrade ◽  
Ana Paula Silva Bueno ◽  
Mário José Romañach ◽  
...  
Keyword(s):  
Latin America ◽  
Head And Neck ◽  
Langerhans Cell Histiocytosis ◽  
Immunohistochemical Study ◽  
Langerhans Cell

scholarly journals Osteoprotegerin-Deficient Male Mice as a Model for Severe Alveolar Bone Loss: Comparison With RANKL-Overexpressing Transgenic Male Mice

Endocrinology ◽  
2013 ◽  
Vol 154 (2) ◽  
pp. 773-782 ◽  
Author(s):  
Masanori Koide ◽  
Yasuhiro Kobayashi ◽  
Tadashi Ninomiya ◽  
Midori Nakamura ◽  
Hisataka Yasuda ◽  
...  
Keyword(s):  
Bone Resorption ◽  
Bone Loss ◽  
Alveolar Bone ◽  
Nuclear Factor Κb ◽  
Alveolar Bone Loss ◽  
Male Mice ◽  
Periodontal Tissues ◽  
Cortical Areas ◽  
Immunohistochemical Analyses ◽  
Transgenic Male

Periodontitis, an inflammatory disease of periodontal tissues, is characterized by excessive alveolar bone resorption. An increase in the receptor activator of nuclear factor-κB ligand (RANKL) to osteoprotegerin (OPG) ratio is thought to reflect the severity of periodontitis. Here, we examined alveolar bone loss in OPG-deficient (OPG−/−) mice and RANKL-overexpressing transgenic (RANKL-Tg) mice. Alveolar bone loss in OPG−/− mice at 12 weeks was significantly higher than that in RANKL-Tg mice. OPG−/− but not RANKL-Tg mice exhibited severe bone resorption especially in cortical areas of the alveolar bone. An increased number of osteoclasts was observed in the cortical areas in OPG−/− but not in RANKL-Tg mice. Immunohistochemical analyses showed many OPG-positive signals in osteocytes but not osteoblasts. OPG-positive osteocytes in the cortical area of alveolar bones and long bones were abundant in both wild-type and RANKL-Tg mice. This suggests the resorption in cortical bone areas to be prevented by OPG produced locally. To test the usefulness of OPG−/− mice as an animal model for screening drugs to prevent alveolar bone loss, we administered an antimouse RANKL antibody or risedronate, a bisphosphonate, to OPG−/− mice. They suppressed alveolar bone resorption effectively. OPG−/− mice are useful for screening therapeutic agents against alveolar bone loss.

Sign in / Sign up

Export Citation Format

Share Document