JICDRO is a UGC approved journal (Journal no. 63927)

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CASE REPORT
Year : 2020  |  Volume : 12  |  Issue : 1  |  Page : 62-66

Langerhans cell histiocytosis of the mandible – A rare case report


Department of Oral Medicine and Radiology, Sri Ramakrishna Dental College and Hospital, Coimbatore, Tamil Nadu, India

Date of Submission16-Oct-2019
Date of Acceptance18-Nov-2019
Date of Web Publication29-Jul-2020

Correspondence Address:
Dr. Vennila Parameswaran
Department of Oral Medicine and Radiology, Sri Ramakrishna Dental College and Hospital, SNR College Road, Nava India, Peelamedu, Coimbatore - 641 004, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jicdro.jicdro_50_19

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   Abstract 


Langerhans cell histiocytosis (LCH) is a rare idiopathic disease that is characterized by the clonal proliferation of bone marrow-derived Langerhans cells. It usually occurs in children at an incidence of 2.6–5.4/10,00,000 children/year. The disease occurs with a slight male predilection. The peak age ranges from 1 to 4 years. The common oral findings include the gingival enlargement, oral ulcers, and loosening of teeth. The variable radiographic appearance mimicking odontogenic cyst, osteomyelitis, or a malignancy poses a diagnostic challenge. This rare case report emphasizes on the differential diagnosis of LCH.

Keywords: Eosinophilic granuloma, immunohistochemistry, Langerhans cell histiocytosis, management, radiographic features


How to cite this article:
Parameswaran V, Bano S. Langerhans cell histiocytosis of the mandible – A rare case report. J Int Clin Dent Res Organ 2020;12:62-6

How to cite this URL:
Parameswaran V, Bano S. Langerhans cell histiocytosis of the mandible – A rare case report. J Int Clin Dent Res Organ [serial online] 2020 [cited 2020 Aug 5];12:62-6. Available from: http://www.jicdro.org/text.asp?2020/12/1/62/291112




   Introduction Top


Langerhans cell Histiocytosis formerly known as histiocytosis X,[1] is an uncommon disorder that is characterized by the nodules composed of heterogeneous cell population.[2] The clinical manifestation of LCH varies from a single organ disease that could spontaneously go to remission to a systemic and aggressive disease that can lead to death. Any organ can be involved, but bone and skin have a higher frequency of involvement.[3]

The radiological signs of LCH in cases of jaw involvement present as floating teeth. The skull lesion presents as punched-out areas with beveled edges geographic skull.[4] The cytoplasmic immune staining shows the presence of Birbeck granules. Treatment modalities include multiagent chemotherapy and immunosuppressant therapy, surgery, and radiation therapy.[5]


   Case Report Top


A 34-year-old male patient reported to the department of oral medicine and radiology with a chief complaint of pain in the lower front tooth region for the past 1 month.

The patient was apparently normal 2 months back after which he experienced a sudden onset of pricking type of pain in lower jaw that is intermittent, nonradiating, and aggravating on touch/pressure. Dental history revealed root canal treatment done before 2 months for a lower front tooth.

On extraoral examination, there was tenderness over the inferior border of the mandible from 34 to 44 region [Figure 1].
Figure 1: frontal view - extraoral image with no facial asymmetry

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On Intra oral examination, there was no evident clinical finding [Figure 2].
Figure 2: intraoral view revealing no evident clinical finding

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Orthopantomograph was advised which revealed a well-defined osteolytic lesion with scalloped borders present that extended from the mesial surface of 36 to the mesial surface of 46 with impacted 33 [Figure 3].
Figure 3: OPG showing expansile osteolytic lesion in the anterior mandible with impacted 33

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The panoramic section of cone-beam computed tomography (CT) revealed a well-defined osteolytic lesion in the anterior mandible extending from 35 to 45 region with radiopaque septations surrounding impacted 33. Discontinuity was noted in the anterior aspect of inferior border of the mandible [Figure 4] and [Figure 5].
Figure 4: skull radiograph

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Figure 5: radiograph of long bones

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Based on the findings, the radiographic differential diagnosis of odontogenic keratocyst (OKC), ameloblastoma, and calcifying epithelial odontogenic cyst (CEOC) was given.

Radiograph of skull, long bones, and ribs was advised which showed multiple punched-out lesions [Figure 6], [Figure 7], [Figure 8].
Figure 6: AP view of the chest

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Figure 7: CBCT image – panoramic section

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Figure 8: CBCT image – sagittal section Figures 7 and 8 showing osteolytic lesion in the anterior mandible with radiopaque septations

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Incisional biopsy showed the presence of sheets of round to polyhedral cells with pale eosinophilic cytoplasm with ill-defined cytoplasmic borders suggestive of “LCH/eosinophilic granuloma” [Figure 9].
Figure 9: histopathological image: Langerhans cells showing folded/grooved nuclei with fine chromatin and unremarkable nucleoli (H and E, ×400

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Immunohistochemistry showed positive S100 and CD1a markers which confirmed the final diagnosis of LCH [Figure 10] and [Figure 11].
Figure 10: immunohistochemistry image: CD1a marker showing membrane positivity

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Figure 11: immunohistochemistry image: S100 marker showing nuclear and cytoplasmic expression

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   Discussion Top


The nomenclature-histiocytosis X was termed by Lichtenstein in 1953 to describe three clinical varieties which showed some histological characteristics in common eosinophilic granuloma, Letterer–Siwe syndrome, and Hand–Schüller–Christian syndrome.[6]

The etiology of LCH is idiopathic but various theories such as environment, infection, genetics, and immunology are suggested.[6]

The rate of incidence is more common in children with peak of 1–3 years.[7] LCH occurs twice more common in the mandible than the maxilla with predilection in the posterior region.[8] It occurs with a slight male predilection which is similar to our case.

LCH involves the head and neck region quite commonly and in particular the bones of the skull and jaws. Gingiva and hard palate are the most commonly affected sites in maxillomandibular involvement.[9] Swelling and pain are the most common findings of LCH. Other symptoms such as ulceroproliferative lesions, bleeding of gingiva, mobility of teeth, floating teeth, sore mouth, jaw pain, facial swelling, delayed healing, and mental nerve paraesthesia are also reported in the literature.[6] In contrast, this present case reported only with pain of lower jaw with the absence of swelling/expansion.

According to literature, radiographically, LCH jaw lesions are divided into alveolar and intraosseous types.

The intraosseous variety presents in the ramus of the mandible as solitary well-defined oval/round radiolucent area. LCH destroys bone, including lamina dura, and as a result, the teeth appear “standing in space.” The alveolar variety represents multiple areas of “scooped out shape of bone destruction.” These lesions also stimulate periosteal new bone formation.[8]

In the skull, the lesion appears as punched-out areas with beveled edges depicting geographic skull,[4] which was present in our case.

According to literature, CT findings present as well-circumscribed osteolytic unilocular/multilocular cystic lesion with periosteal reaction.[10]

Aggressive lesion presents as areas of multiple irregular bony destruction with noncorticated border involving buccal and lingual cortical plates.[10]

In the present case, periosteal reaction was not observed.

Since punched-out skull lesions were present, OKC, ameloblastoma, and CEOC were ruled out.

As LCH lacks pathognomonic clinical and radiographic characteristics, a definitive diagnosis should be based on histological and immunohistochemical examination. Microscopically, LCH shows Langerhans cells (immature dendritic cells) with abundant eosinophilic cytoplasm,[9] which was present in our case.

Because monoclonal proliferation of CD1-positive histiocytes has been shown in all forms of LCH, and the immunostaining for CD 1a is a routine procedure for diagnostic confirmation of LCH.[11] In our case, CD 1a immunostaining was positive and hence, confirmatory for LCH.

The treatment of LCH depends upon lesion size, degree of tissue involvement, and age of the patient. Therapy includes surgery, radiation, chemotherapy, or other individually or in combination.[11] More recently, intralesional injections of corticosteroids such as prednisone (20–30 mg Once a day (OD) for 2–4 weeks, followed by tapering of the doses) have been found to offer good results.[12] Surgical curettage and steroid injections are given in case of solitary jaw lesions. Excision followed by reconstruction was done in severe cases.[13] In our case, the patient presented with a disseminated lesion, and hence chemotherapy (methotrexate and vinblastine with prednisone) is to be delivered.

The prognosis of LCH is favorable. The course of disease is unpredictable and evolves with multiple reactivations. Prognosis for LCH is the most favorable, although it depends on age of presentation and extent of vital organ involvement.[5] Hence, recurrence rate ranges from 1.6% to 25%, and the patient should be closely followed up for a long period to ensure remission.[11] The patient has lost follow-up in our case.

To conclude, occurrence of LCH in older adults is exceptional. The most expansile osteolytic lesions associated with impacted tooth with occurring in the anterior mandible include OKC, ameloblastoma, and calcifying epithelial odontogenic tumor. LCH, a nonodontogenic bony disorder, can be considered as a radiographic differential diagnosis for such odontogenic cysts and tumors. This article emphasizes on the enhancement of understanding of similar diverse clinical presentations which can be overlooked by the dentist in diagnosis and decision-making process.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgement

Dr. J Dinakar Principal and Head of the Department Oral Pathology and Microbiology Sri Ramakrishna Dental College and Hospital.

Financial support and sponsorship

Nil.

Conflicts of interest

None.



 
   References Top

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Altay MA, Sindel A, Ozalp O, Kocabalkan B, Ozbudak İH, Erdem R, et al. Langerhans cell histiocytosis: A diagnostic challenge in the oral cavity. Case Rep Pathol 2017;2017:1-6.  Back to cited text no. 9
    
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Lee BD, Lee W, Lee J, Son HJ. Eosinophilic granuloma in the anterior mandible mimicking radicular cyst. Imaging Sci Dent 2013;43:117-22.  Back to cited text no. 10
    
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Yashoda-Devi B, Rakesh N, Agarwal M. Langerhans cell histiocytosis with oral manifestations: A rare and unusual case report. J Clin Exp Dent 2012;4:e252-5.  Back to cited text no. 11
    
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Rao DG, Trivedi MV, Havale R, Shrutha SP. A rare and unusual case report of Langerhans cell histiocytosis. J Oral Maxillofac Pathol 2017;21:140-4.  Back to cited text no. 12
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Camargo AJ, Cheade M, Martinelli C, Monteiro SA, de Oliveira Santos C, Watanabe PC. Langerhans cell histiocytosis (histiocytosis x)-in the mandible. Braz Dent Sci 2013;16:93-8.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]



 

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