scholarly journals Periodontal manifestations of Langerhans cell histiocytosis: a systematic review

2021 ◽  
Author(s):  
Julia C. Difloe-Geisert ◽  
Selina A. Bernauer ◽  
Noémie Schneeberger ◽  
Michael M. Bornstein ◽  
Clemens Walter
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Case Series ◽  
Langerhans Cell ◽  
Treatment Modalities ◽  
Bone Lesions ◽  
Bias Assessment ◽  
Initial Symptoms ◽  
Treatment Measures ◽  
Oral Symptoms ◽  
Tooth Extractions

Abstract Objectives To explore the evidence of periodontal manifestations and treatment modalities in patients with Langerhans cell histiocytosis (LCH). Material and methods A systematic literature search was performed and the criteria for PRISMA and risk of bias assessment were applied. Human clinical studies (≥10 patients) presenting patients with LCH and periodontal findings were considered for inclusion. Results From 298 titles identified, six case series with a total of 1278 patients suffering from LCH were included. In these studies, oral symptoms were reported in a frequency ranging from 10 to 100%. Overall, in 216 patients (17%), oral symptoms were observed. Out of these patients, 49–100% demonstrated periodontal symptoms. The most common oral findings were pain, swelling, tooth loss/mobility, and bone lesions. Specific periodontal findings comprised varying frequencies of gingival ulcerations, increased pocket depths, and gingival bleeding. Treatment measures constituted of surgical curettage of bone lesions, soft tissue excision and/or tooth extractions, radiotherapy, systemic chemotherapy, or a combination of these approaches. Healing without recurrence of oral lesions was reported in most of the cases. Conclusions The available evidence on periodontal manifestations in LCH patients is heterogeneous. Several oral and periodontal findings were reported and may occur as initial symptoms and/or at later stages of the disease. Clinical relevance The dentist should be aware of possible oral involvement of systemic diseases such as LCH, and these manifestations may mimic periodontal disease.

scholarly journals Initial Symptoms of Langerhans Cell Histiocytosis: A Case Series

2019 ◽  
Vol 6 ◽  
pp. 2333794X1985737 ◽  
Author(s):  
Yukari Atsumi ◽  
Yuya Saito ◽  
Hiroshi Hataya ◽  
Yuki Yuza
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Medical Center ◽  
Bone Lesion ◽  
Case Series ◽  
Langerhans Cell ◽  
Long Bone ◽  
Bone Lesions ◽  
Initial Symptom ◽  
Initial Symptoms ◽  
Skull Lesion

Langerhans cell histiocytosis (LCH) is a rare childhood hematopoietic disease, and hence, there are few reports summarizing the course leading to the diagnosis. We described the initial symptoms and the clinical course of LCH. We carried out a retrospective review of charts from a single medical center, and 21 patients with the diagnosis of LCH were enrolled. The initial symptoms of 16 cases were caused by bone lesions; of these cases, there were 8 instances of soft tissue swelling as the initial symptom (38%) and 8 instances of bone pain without swelling (38%). Among the cases of bone lesion, 4 of 6 cases of skull lesion were painless while all vertebral body lesions and long bone lesions were accompanied by pain. LCH bone lesions caused various symptoms depending on the site of the lesion and this makes the diagnosis difficult. A detailed physical examination and imaging studies are recommended for early diagnosis.

Treatment of Langerhans cell histiocytosis bone lesions with zoledronic acid: a case series

2011 ◽  
Vol 93 (6) ◽  
pp. 782-786 ◽  
Author(s):  
Shanthi Sivendran ◽  
Harold Harvey ◽  
Allan Lipton ◽  
Joseph Drabick
Keyword(s):  
Zoledronic Acid ◽  
Langerhans Cell Histiocytosis ◽  
Case Series ◽  
Langerhans Cell ◽  
Bone Lesions

scholarly journals BISPHOSPHONATES IN LANGERHANS CELL HISTIOCYTOSIS: AN INTERNATIONAL RETROSPECTIVE CASE SERIES

2016 ◽  
Vol 8 ◽  
pp. 2016033 ◽  
Author(s):  
Deepak Chellapandian ◽  
Polyzois Makras ◽  
Gregory Kaltsas ◽  
Cor Van den Bos ◽  
Lamia Naccache ◽  
...  
Keyword(s):  
Adverse Effects ◽  
Langerhans Cell Histiocytosis ◽  
Case Series ◽  
Initial Response ◽  
Langerhans Cell ◽  
Bone Lesions ◽  
International Studies ◽  
First Line ◽  
Retrospective Case ◽  
Multisystem Disease

Background: Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH), which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH. Method: We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation. Results: Ten patients (77%) had a single system bone disease, and 3 (23%) had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years). Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve  (92%) achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years). One patient did not respond. No major adverse effects were reported in this series.  Conclusion: Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warranted.

scholarly journals Isolated Bone Lesions in the Mandible and Maxilla of Langerhans Cell Histiocytosis Treated with Fractionated Stereotactic Low-Dose Radiotherapy: Case Report and 5-Year Follow-Up

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Antoine Berberi ◽  
Georges Aoun ◽  
Georges Aad ◽  
Elie Azar
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Low Dose ◽  
Healing Process ◽  
Langerhans Cell ◽  
Treatment Modalities ◽  
Bone Lesions ◽  
Entire Body ◽  
Low Dose Radiotherapy ◽  
Custom Made ◽  
The Right

Langerhans cell histiocytosis is a benign histiocytic disorder touching both genders and can occur at any age. It is currently classified by the Histiocyte Society as an inflammatory myeloid neoplasm of mixed cellularity. Clinically, it is illustrated by single or multiple osteolytic bone lesions associated with ulceration of the skin and soft tissues. Disease outcome is highly variable, depending on the degree of involvement. Bone pathologies are observed in 60% of cases as uni- or multifocal lesions. Several treatment modalities have been proposed and include surgical excision, intralesion steroid injection, chemotherapy, and low-dose radiotherapy. In this paper, we report a case of a 42-year-old male suffering from gingival swelling in the left side of his mandible and the right side of the maxilla. Clinical, radiological, and histological examinations confirm the diagnosis of Langerhans cell histiocytosis. Hematological investigation, entire body CT scan, and bone scintigraphy confirmed the limitation of the lesions in the right maxilla and on the left mandible. The lesions were treated with fractionated stereotactic low-dose radiotherapy, 24 Gy in 16 fractions, by using a linear accelerator with a custom-made rigid mask for accurate immobilization of the head with confirmed precision, which allows noninvasive approaches. Complete remission was achieved clinically and radiologically after one year, and a panoramic X-ray after 5 years confirms the bone healing process. Fractionated stereotactic low-dose RT could be adopted as an effective treatment.

scholarly journals Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
B. Routy ◽  
J. Hoang ◽  
J. Gruber
Keyword(s):  
Smoking Cessation ◽  
Cigarette Smoking ◽  
Langerhans Cell Histiocytosis ◽  
Case Series ◽  
Bone Destruction ◽  
Bone Diseases ◽  
Langerhans Cell ◽  
Braf V600e ◽  
Bone Lesions ◽  
Pulmonary Langerhans Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH) presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8) vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented.

scholarly journals Paediatric Langerhans Cell Histiocytosis Disease: Long-Term Sequelae in the Hypothalamic Endocrine System

2021 ◽  
pp. 1-9
Author(s):  
Elisa Vaiani ◽  
Guido Felizzia ◽  
Fabiana Lubieniecki ◽  
Jorge Braier ◽  
Alicia Belgorosky
Keyword(s):  
Anterior Pituitary ◽  
Langerhans Cell Histiocytosis ◽  
Endocrine System ◽  
Langerhans Cell ◽  
Hormone Deficiency ◽  
Bone Lesions ◽  
Pituitary Hormone ◽  
Multisystem Disease ◽  
Anterior Pituitary Hormone

Langerhans cell histiocytosis (LCH) is a disorder of the mononuclear phagocyte system that can affect almost any organ and system. The most common central nervous system (CNS) manifestation in LCH is the infiltration of the hypothalamic-pituitary region leading to destruction and neurodegeneration of CNS tissue. The latter causes the most frequent endocrinological manifestation, that is, central diabetes insipidus (CDI), and less often anterior pituitary hormone deficiency (APD). The reported incidence of CDI is estimated between 11.5 and 24% and is considered a risk factor for neurodegenerative disease and APD. Three risk factors for development of CDI are recognized in the majority of the studies: (1) multisystem disease, (2) the occurrence of reactivations or active disease for a prolonged period, and (3) the presence of craniofacial bone lesions. Since CDI may occur as the first manifestation of LCH, differential diagnosis of malignant diseases like germ cell tumours must be made. APD is almost always associated with CDI and can appear several years after the diagnosis of CDI. Growth hormone is the most commonly affected anterior pituitary hormone. Despite significant advances in the knowledge of LCH in recent years, little progress has been made in preventing long-term sequelae such as those affecting the hypothalamic-pituitary system.

A Review of Noninfectious Diseases Masquerading as Acute Mastoiditis

2021 ◽  
pp. 019459982110641
Author(s):  
Kevin Wong ◽  
Annie E. Arrighi-Allisan ◽  
Caleb J. Fan ◽  
George B. Wanna ◽  
Maura K. Cosetti ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Inflammatory Diseases ◽  
Case Series ◽  
Diagnostic Algorithm ◽  
Langerhans Cell ◽  
Myelogenous Leukemia ◽  
Common Disease ◽  
Acute Mastoiditis ◽  
Significant Group ◽  
Presenting Symptoms

Objective Acute mastoiditis is commonly attributed to infection. Rarely do clinicians encounter cases that do not respond to traditional antibiotics or surgical management. The goal of this study was to systematically review the literature to characterize diseases masquerading as acute infectious mastoiditis. Data Sources PubMed, Embase, and Scopus. Review Methods A systematic review was performed to identify all publications that reported on diseases with presentations mimicking acute mastoiditis, defined as postauricular redness, swelling, and tenderness. We included clinical prospective studies, retrospective studies, and case series/reports. Exclusion criteria included non-English articles, letters/commentaries, abstracts, and review articles. Results Out of 3339 results, 35 studies met final inclusion criteria. In children, 11 diseases were reported to mimic mastoiditis, including solid tumors, hematologic diseases, and autoimmune/inflammatory diseases. The most common disease in children was Langerhans cell histiocytosis, followed by rhabdomyosarcoma and acute myelogenous leukemia. In adults, 8 additional diseases were reported. The most common disease in adults was squamous cell carcinoma, followed by nasopharyngeal carcinoma and Langerhans cell histiocytosis. Presenting symptoms are reviewed, as well as characteristic radiographic, laboratory, and intraoperative features that may assist with diagnosis. A diagnostic algorithm for atypical cases of acute mastoiditis is proposed. Conclusion A small but significant group of diseases in children and adults can mimic acute mastoiditis. In such cases, history and examination alone may be insufficient to reach a diagnosis, and further investigation may be necessary. Otolaryngologists should always be mindful of the possibility that noninfectious pathologies may present with a constellation of symptoms similar to mastoiditis.

scholarly journals Langerhans Cell Histiocytosis with Isolated Central Diabetes Insipidus, Low Grade Fever and Sellar Erosion

2021 ◽  
Author(s):  
İclal Okur ◽  
Hasan Ari ◽  
Semra Çetinkaya ◽  
Betül Emine Derinkuyu ◽  
Gizem Çağlar ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Bone Structure ◽  
Sella Turcica ◽  
Langerhans Cell ◽  
Central Diabetes Insipidus ◽  
Bone Lesions ◽  
Warning Sign ◽  
Low Grade ◽  
First Case

Langerhans cell histiocytosis (LCH) is a rare disease of the monocyte-macrophage system. Although it is known that bone involvement is seen very frequently in cases with LCH, our case is the first case with a lytic-destructive lesion in the bone structure forming sella turcica. A 4-year-old, 5-month-old male patient who applied to our outpatient clinic was diagnosed with Langerhans cell histiocytosis in further examination after the diagnosis of central diabetes insipidus (CDI) was made. On cranial magnetic resonance imaging (MRI), widespread lytic-destructive bone lesions were observed in the bone structure forming the sella (sphenoid bone), sellar destruction not previously described in the literature. Sellar erosion has not been reported before in cases diagnosed with LCH in the literature. The presence of low-grade fever in a patient presenting with isolated CDI is a warning sign for the diagnosis of LCH.

scholarly journals Langerhans-Cell Histiocytoses - Epidemiology, Classification, Clinical Features, Diagnosis, Complications, Treatment and Prognosis

2016 ◽  
Vol 9 (1) ◽  
pp. 3-16 ◽  
Author(s):  
Vera E. Papochieva ◽  
Dimitrinka S. Miteva ◽  
Penka I. Perenovska ◽  
Guergana Petrova
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Eosinophilic Granuloma ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Bone Lesions ◽  
Multisystem Disease ◽  
The Past ◽  
System Disease ◽  
Young Smokers ◽  
Underlying Pathology

Summary Histiocytoses comprise a group of diverse diseases of unknown etiology with various clinical presentation and evolution. The underlying pathology is characterised by accumulation and infiltration of variable numbers of cells of the monocyte-macrophage line in the affected tissues and organs. Histiocytoses are divided into three major classes: Langerhans cell histiocytosis (LCH), non- Langerhans cell histiocytosis, and malignant histiocytic disorders. The term LCH (also known in the past as histiocytosis X) encompasses the following rare diseases: Eosinophilic Granuloma, Hand-Schuller-Christian disease, Letterer-Siwe disease, Hashimoto-Pritzker disease, in which accumulation of pathologic Langerhans cells (LCs) leads to tissue damage. LCs usually reside in the skin and ensure protection against infections by destroying foreign substances. LC accumulation is caused by antigen stimulation and inadequate immune response. Thus, clinical LCH manifestations range from isolated disease with mono- or multifocal bone lesions to disseminated multisystem disease. LCH is a rare disease, affecting mainly children and young smokers, aged 20-50 years. Lung involvement in LCH usually presents as a mono-system disease and is characterized by Langerhans cell granulomas (LCG) infiltrating and impairing the distal bronchioles. The definite diagnosis is based on lung biopsy of CAT selected LCG areas. So far, there is no an effective treatment, but the better understanding of the mechanisms involved in the pathogenesis of the disease would help in the development of effective therapeutic strategies in the future.

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