Langerhans Cell Histiocytosis in the Oral Cavity (original) (raw)
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A series of 13 new cases of langerhans cell histiocytosis of the oral cavity: a master of disguise
Oral Health and Care, 2017
Langerhans cell histiocytosis(LCH) is a disease characterized by clonal proliferation and excessive accumulation of disseminated forms of dendritic Langerhans cells. The aims of thestudy were to describe clinico-pathological presentations of thirteen new cases of LCH of the oral cavity and to present its differential diagnoses. The majority of 61.53% (8/13) of cases occurred in infants and adolescents with female predominance (61.53%). Out of all the lesions,61.53 % occurred intraosseously;with majority 53.82% (7/13) presenting on the posterior mandible and 15.38% (2/13)in the anterior mandible.Five lesions extended to involve more than one site. Radiologically all intraosseous lesions presented as osteolytic lesions with ill-defined or well defined margins. A wide range of clinical differential diagnoses including malignancies have been proposed indicating the aggressive clinical presentations exhibited by some lesions. Histopathologically, all lesions at first glance resembled an infection/chronic inflammatory process and contained mixed inflammatory cells including eosinophils. Deep fungal infections and traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) were the two main histopathological differential diagnoses that were considered for hard tissue and soft tissue lesions respectively. Round mononuclear cells, containing eosinophilic cytoplasms and peripheral indented cerebriform nuclei were only observed under higher magnification. Immunohistochemical investigations with S-100 antibody reveled nuclear and cytoplasmic positivity in mononuclear cells confirming these cells as Langerhans cells. All the patients were treated with complete surgical excision with or without chemo/radiotherapyand are under follow up care. In conclusion, as LCH of oral cavity may have a broad spectrum of clinico-pathological presentations, high degree of clinical awareness is required to arrive at the definitive diagnosis. The histopathological diagnosis is reliable with immunohistochemical confirmation of the Langerhans cell. However, awareness of its differential diagnosis is important to avoid misdiagnosis; resulting in incorrect management.
Langerhans Cell Histiocytosis of the Oral Mucosa: A Rare Case Report
https://www.ijhsr.org/IJHSR\_Vol.7\_Issue.1\_Jan2017/IJHSR\_Abstract.041.html, 2017
Langerhans cell histiocytosis (LCH) is a rare idiopathic disease characterized by the clonal proliferation of bone marrow derived Langerhans cells. It usually occurs in children. Their clinical features simulate common oral findings such as gingival enlargement, oral ulcers, and mobility of teeth, along with nonspecific radiographic features, thus easily leading to the misdiagnosis and therefore, it could be overlooked by dentists. We report a rare case of LCH in 38-year-old adult male, characterized by oral mucosal ulcerations with no involvement of the underlying bone. The lesion was histologically proven to be LCH and was confirmed with immunohistochemical staining of S100 protein and CD1a antigen.
Langerhans cell histiocytosis (LCH) is a rare hematological disorder characterized by local or generalized, uncontrolled proliferation and infiltration of Langerhans type of histiocytic cells. It represents a spectrum of clinicopathologic disorders, ranging from a highly aggressive and frequently fatal multisystem disease to an easily cured solitary lesion of bone. Involvement of children and the younger age group is more common than the adults. Oral cavity involvement occurs early in LCH, but the initial symptoms are generally nonspecific, often causing misdiagnosis. This report describes a rare case of chronic localized LCH in an adult patient, with involvement of oral cavity. A 34-year-old male patient presented with multiple nodulo-papular, ulcerated lesions in gingiva involving both the jaws (primarily mandible) and the left buccal mucosa, in addition to regional teeth mobility. The most striking feature was that even after extraction of mobile teeth, the lesions persisted. After recording proper history, performing clinical and radiological evaluation, an incisional biopsy was performed followed by histopathology and immunohistochemistry to reach a confirmatory diagnosis of LCH, thereby implementing early and appropriate initiation of treatment.
Langerhans cell histiocytosis with oral manifestations: a rare and unusual case report
Journal of Clinical and Experimental Dentistry, 2012
Langerhans cell histiocytosis (LCH), is a rare, proliferative disorder in which the accumulation of pathologic Langerhans cells leads to local tissue infiltration and destruction. We present a case of a 32 years old, completely edentulous female patient who presented with erythema of hard palate, maxillary alveolar mucosa and mucosa over the distobuccal part of mandibular alveolar ridge with foci of ulcerations. Histopathologic features were suggestive of LCH which was confirmed by immunohistochemistry which was CD1a positive, confirmatory for LCH. Bone scan revealed multiple bone involvement. At this stage, disease had already progressed to multisystem involvement with endocrinal abnormalities (primary hypothyroidism and hyperprolactinemia), requiring aggressive treatment. Therefore, this case is a reminder of the possibility of occurrence of this rare disease in the oral cavity which might manifest itself in multiple presentations thus easily leading to the misdiagnosis and therefore, it could be easily overlooked by dentists.
Solitary langerhans cell histiocytosis in an adult: case report and literature review
BMC Research Notes, 2016
Background: Langerhans cell histiocytosis (LCH) is a disease that often affects children, but can also occur in adults and smokers. Oral manifestations are unusual and are characterized by bone pain, tooth mobility, necrotic ulcers and local edema. The aim of this paper is to describe a clinical case of LCH in an oral cavity that mimicked oral squamous cell carcinoma. Case presentation: A male, 63 years old, complaining about a "wound in the mouth" for 6 months, without any pain or spontaneous bleeding. His medical history was free of disease. The patient was a smoker for 33 years. Intraoral examination revealed a destructive ulcerative lesion around the upper left first and second molars that resembled an oral squamous cell carcinoma. Biopsy of the ulcerative lesion was performed and the microscopic features showed an inflammatory infiltrate rich in plasma cells. Based on this microscopical finding, the final diagnosis was periodontal disease associated with a proliferative non-neoplastic lesion. The patient was referred to a specialized dental surgeon and underwent periodontal therapy including surgical procedures. After that, according to follow-up with the patient, there were no signs of disease remission. The lesion increased in size, although the patient did not complain of any symptoms. A second biopsy was performed and the microscopic features again showed a rich inflammatory infiltrate with mononuclear cells and histiocytic cells, characterized by pale histiocytes with lobed nuclei, resembling a bean. A varying number of eosinophils also were observed, without any evidence of atypical cells present in this infiltrate. An immunohistochemical staining panel was done to determine the nature of this inflammatory infiltrate by using antibodies S-100, CD1a, CD-68 and CD45RO that were positive. These immunohistochemical findings were fundamental for the final diagnosis of LCH. The treatment included surgical extraction of all superior teeth, radiation and systemic corticoid therapies. After 8 years of treatment, the patient is free of disease. Conclusion: Although LCH is an unusual lesion in an oral cavity, it can be present. Biopsy and a histological exam are essential to establish the diagnosis. Immunohistochemicals were fundamental to exclude malignant lesion and to confirm the diagnosis of LCH.
Multisystem Langerhans cell histiocytosis presenting as an oral lesion
Journal of Oral and Maxillofacial Pathology, 2013
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which the pathologic Langerhans cells infiltrate and destroy the tissues. Patients with LCH present varied clinical manifestations. Cutaneous lesions in LCH manifest as vesiculopapular eruptions that often mimic various infectious diseases particularly in infants. We present a case of a female infant with an ulcerative lesion intraorally. The baby was asymptomatic otherwise. A detailed history revealed the presence of cutaneous lesions that was overlooked by her parents. Conclusion: This report tries to briefly discuss the current concepts regarding the etiology of LCH. An attempt has been made to emphasis the need for a through systemic examination. The protocol of investigative procedures to be adopted in LCH is also discussed.
A rare case of oral multisystem Langerhans cell histiocytosis
Journal of Clinical and Experimental Dentistry
Langerhans cell histiocytosis (LCH) is a rare disorder characterized by high proliferation of Langerhans dendritic cells. LCH is a solitary or multifocal disease that primarily involves bone tissue and often affects children and young men. A 29 years-old Caucasian man was referred to the Oral Surgery Unit of George Eastman Hospital-Umberto I teaching hospital, with third degree mobility of teeth belonging to second, third and fourth quadrant. Panoramic radiograph showed multiple radiolucent areas with well demarcated borders on the right and left site of the mandible and on the left site of the maxilla. Extractions of compromised teeth and biopsy of the osteolytic tissue were performed. The final diagnosis of multisystem Langerhans cell histiocytosis of the soft and hard tissues of the oral cavity was made. The patient was sent to the Hematology department of Umberto I Teaching Hospital of "Sapienza"-University of Rome for the proper treatment. The present case of rare multisystem LCH involving oral hard and soft tissues shows the strong importance of better investigate, with appropriate additional exams, initial shifty symptoms that could lead to a misdiagnosis.
Oral Oncology Extra, 2006
A 2.5 years old boy was referred to the Department of oral medicine because of substantial erythema and ulcerations on palatal, buccal and alveolar regions. Unfortunately, it took two subsequent visits to our Department in sixmonths intervals, and re-evaluation at Pediatric oncology clinic where diagnosis of Langerhans cell histiocytosis (LCH) was finally established. At that stage, disease already progressed to multisystem life-threatening presentation, requiring aggressive treatment. Therefore, this case is a reminder of the possibility of occurrence of this rare disease in the oral cavity which might manifest itself in multiple presentations thus easily leading to the misdiagnosis and therefore it could be easily overlooked by dentists.
Recurrence of Langerhans Cell Histiocytosis of Jaws: A Case Report with One Year Follow-up
2019
Abstract: Langerhans cell histiocytosis (LCH) is an unusual disorder characterized by high proliferation of Langerhans cells. A 27-year-old male patient was referred to the Oral Medicine Clinic, Faculty of Dentistry, Altinbas University with a complaint of wound that does not heal for eight months. The clinical examination showed palatal mucosal ulceration extending to the bone at the upper right first molar region and ulceration at the lingual mucosa of the lower right first molar. The patient had been previously evaluated by a different dentist and was told that it was an aphthous lesion. A punch biopsy from the palatal mucosa and alveolar mucosa near the lesion revealed Langerhans cells and positive reaction to CD1 so the patient was diagnosed with LCH. The insignificant treatment trials may lead to diagnostic delay. Oral lesions may be the earliest symptom of LCH and in most cases; the oral cavity may be the only area involved. Awareness of lesions in the oral mucosa is importan...
A rare and unusual case report of Langerhans cell histiocytosis
Journal of Oral and Maxillofacial Pathology
Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, is an uncommon hematological disorder affecting infants and young children. It is the condition characterized by uncontrolled stimulation and proliferation of normal antigen presenting cells, Langerhans cells. Although because of its relatively low incidence, limited data are available regarding the epidemiology of LCH, with estimation of 2-5 cases per million inhabitants per year in the year 2009. [1] The diagnosis of LCH is mainly based on histopathological examination, and the disease is broadly categorized into three disorders based on the clinical presentations, each of which is staged clinically according to Greenberg et al. clinical staging system. [1] Oral involvements are commonly the first manifestation in LCH, but the initial symptoms are generally nonspecific, causing misdiagnoses. [1] The purpose of reporting this case is to discuss clinical and radiological features of LCH and role of dentists in diagnosing and managing such lesions. Management and prognosis of LCH are based on the clinical presentation of the lesion.