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.

Monostotic Langerhans' Cell Histiocytosis in a Child with Central Diabetes Insipidus

2012 ◽  
Vol 36 (4) ◽  
pp. 377-381 ◽  
Author(s):  
E Costa Studart Soares ◽  
ARP Quidute ◽  
FW Gurgel Costa ◽  
MH Costa Gurgel ◽  
AP Negreiros Nunes Alves ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Reticuloendothelial System ◽  
Langerhans Cell ◽  
Central Diabetes Insipidus ◽  
Important Indicator ◽  
Presenting Symptoms ◽  
Dental Practitioners ◽  
Multi Agent ◽  
Underlying Diseases

Langerhans' cell histiocytosis (LCH) comprises a rare group of reticuloendothelial system disorders that can produce focal or systemic manifestations. Diabetes insipidus is considered to be an important indicator of serious underlying diseases in children, including LCH. We report the case of a young patient with monostotic LCH confined to the mandibular ramus, who was diagnosed with the disease after presenting symptoms of central diabetes insipidus and was satisfactorily treated with multi-agent chemotherapy. Additionally, we discuss the clinical, radiographic, histological and immunohistochemical findings, as well as the multidisciplinary approach of this important disease, which should receive attention by dental practitioners, especially when it occurs in children.

Adult Langerhans cell histiocytosis: A contemporary single-institution series of 186 patients.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 7018-7018
Author(s):  
Gaurav Goyal ◽  
Marie Hu ◽  
Jason R Young ◽  
Robert Vassallo ◽  
Jay H Ryu ◽  
...  
Keyword(s):  
Smoking Cessation ◽  
Langerhans Cell Histiocytosis ◽  
Overall Response Rate ◽  
Langerhans Cell ◽  
Braf V600e ◽  
First Line ◽  
Multisystem Disease ◽  
Presenting Symptoms ◽  
Systemic Therapies

7018 Background: Langerhans cell histiocytosis (LCH) is a rare histiocytic neoplasm driven by MAPK-ERK mutations in majority of patients. Contemporary data on treatments and outcomes in adult LCH are lacking. Hence, we undertook this study to analyze a large cohort of adult LCH patients. Methods: This was a retrospective study of adult (≥18 years) LCH patients seen at our institution between 1998 and 2018. Results: We included 186 patients with adult LCH (median age 43; 19-88), and 54% were females. 70% of patients were diagnosed after 2007. Common presenting symptoms were cough/dyspnea (30%), rash (17%), pain/swelling in head (17%), and diabetes insipidus (10%). 70 (38%) patients had multisystem LCH, 62 (33%) had isolated pulmonary LCH, and 35 (19%) had unifocal LCH. Common sites of involvement included lung (59%), bone (37%), skin (21%), and nervous system (16%). 121 (65%) were smokers; 48% of these had lung disease, while 52% had multisystem disease. 18 of 31 tested (58%) patients had BRAF-V600E mutation. Most common first-line treatment was smoking cessation in 24 patients, and led to an overall response rate (ORR) of 83% in pulmonary lesions. Radiation therapy was used in 11 patients, and led to an ORR 82%. Surgical resection of lesion was done in 23 patients, with relapses in 24%. Systemic therapies were used in 78 (42%) patients (Table). Most common first-line systemic therapy was cladribine with ORR of 78%. Vemurafenib was used in 3 patients with BRAF-V600E, leading to an ORR of 67% . After a median follow-up of 23 months (0-261), 21 patients had died. Of these, 10 died of progressive LCH. Median OS was not reached, and mean OS was 196 months. Conclusions: This is the largest contemporary series of adult LCH. It shows that diverse clinical spectrum, ranging from benign course to a progressive multisystem disease. Although smoking cessation was an effective treatment for pulmonary LCH, a large subset required systemic chemotherapy. [Table: see text]

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

Langerhans cell histiocytosis in adults: a retrospective, single-center case series

2021 ◽  
Author(s):  
Stefano Chiaravalli ◽  
Andrea Ferrari ◽  
Luca Bergamaschi ◽  
Nadia Puma ◽  
Giovanna Gattuso ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Case Series ◽  
Langerhans Cell ◽  
Single Center

Langerhans cell histiocytosis mimicking molluscum contagiosum: A case series

2018 ◽  
Vol 65 (7) ◽  
pp. e27047 ◽  
Author(s):  
José Manuel Fernández Armenteros ◽  
Nuria Arco Huguet ◽  
Veronica Sanmartin Novell ◽  
Felip Vilardell Villellas ◽  
Ana Velasco Sanchez ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Case Series ◽  
Molluscum Contagiosum ◽  
Langerhans Cell

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 Langerhans cell histiocytosis

Blood ◽  
2020 ◽  
Vol 135 (16) ◽  
pp. 1319-1331 ◽  
Author(s):  
Carlos Rodriguez-Galindo ◽  
Carl E. Allen
Keyword(s):  
Organ Dysfunction ◽  
Langerhans Cell Histiocytosis ◽  
Survival Rates ◽  
Local Treatment ◽  
Case Series ◽  
Mapk Signaling ◽  
Langerhans Cell ◽  
Multisystem Disease ◽  
Case Series Report ◽  
Early Case

Abstract Langerhans cell histiocytosis (LCH) is caused by clonal expansion of myeloid precursors that differentiate into CD1a+/CD207+ cells in lesions that leads to a spectrum of organ involvement and dysfunction. The pathogenic cells are defined by constitutive activation of the MAPK signaling pathway. Treatment of LCH is risk-adapted: patients with single lesions may respond well to local treatment, whereas patients with multisystem disease require systemic therapy. Although survival rates for patients without organ dysfunction is excellent, mortality rates for patients with organ dysfunction may reach 20%. Despite progress made in the treatment of LCH, disease reactivation rates remain above 30%, and standard second-line treatment is yet to be established. Treatment failure is associated with increased risks for death and long-term morbidity, including LCH-associated neurodegeneration. Early case series report promising clinical responses in patients with relapsed and refractory LCH treated with BRAF or MEK inhibitors, although potential for this strategy to achieve cure remains uncertain.

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.

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