Premature Eruption Of Tooth: An Unusual Oral Manifestation In A Case Of Multisystem Langerhans Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is a diagnostic and therapeutic challenge. We report on a rare case of its primary oral manifestation that was treated successfully with the BRAF-specific agent, vemurafenib, after insufficient standard LCH treatment. This case underlines the importance of proper diagnosis and the evaluation of targeted therapy as a valuable tool in LCH treatment. Furthermore, the close collaboration of surgeons, oncologists, and dentists is mandatory to ensure adequate treatment, restore the stomatognathic system in debilitating post-treatment situations, improve quality of life, and ensure effective disease control in infants and young patients.

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

BMJ Case Reports ◽

10.1136/bcr-2019-229483 ◽

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.

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Rare Langerhans cell histiocytosis in children: a case report

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20191354 ◽

2019 ◽

Vol 7 (4) ◽

pp. 1367

Author(s):

Lina Fadil ◽

Emtenan Almajid

Keyword(s):

Langerhans Cell Histiocytosis ◽

Plasma Cells ◽

Oral Lesion ◽

Langerhans Cell ◽

Tissue Destruction ◽

Total Period ◽

Oral Manifestation ◽

Initial Symptoms ◽

Good General Condition

Langerhans cell histiocytosis (LCH) is a rare disease, formally known as histiocytosis X that is characterized by abnormal proliferation of histiocytes derived from bone marrow (Langerhans cells), joined with leucocytes, eosinophils, neutrophils, lymphocytes, plasma cells and giant multi-nucleated cells causing tissue destruction. One of the first signs of LCH is oral manifestation, in some cases, the oral cavity may be the only affected area. With the chance of oral lesion incidence in LCH being 77%.Initial symptoms are generally nonspecific, which can easily cause misdiagnoses.The purpose of reporting this case is to discuss the features of LCH clinically and radiographically and in the role of the dentist when diagnosing such lesions for a proper management.An 11-year-old boy reported a complaint of swelling in the left side of the lower jaw that is asymptomatic and had been gradually increasing in size for the past 6 months without any improvements. After preforming a biopsy and diagnosing the lesion as LCH, the patient was then treated with a dose of vinblastine (6 mg/m2 intravenous bolus) for 24 weeks as a total period. Two years follow up; the patient showed no sign of recurrence and is in good general condition. In conclusion, reporting this case serves as documentation of the proper route of clinical assessment and diagnosis of LCH with the best possible treatment as guidance.

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Periodontal disease as the initial oral manifestation of langerhans cell histiocytosis - a case report

Annals of Dentistry ◽

10.22452/adum.vol16no1.5 ◽

2009 ◽

Vol 16 (1) ◽

pp. 31-36

Author(s):

A.R. Ahmadi ◽

S.M. Ismaij ◽

R.B. Zain ◽

Z. Mohamad Zaini

Keyword(s):

Case Report ◽

Periodontal Disease ◽

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Oral Manifestation

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Oral manifestation of Langerhans cell histiocytosis: a case report

BMC Oral Health ◽

10.1186/s12903-018-0568-5 ◽

2018 ◽

Vol 18 (1) ◽

Cited By ~ 7

Author(s):

Julia Luz ◽

Daniel Zweifel ◽

Martin Hüllner ◽

Marco Bühler ◽

Martin Rücker ◽

...

Keyword(s):

Case Report ◽

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Oral Manifestation

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Langerhans Cell Histiocytosis Presenting as a Unifocal Eosinophilic Granuloma in the Body of the Mandible: A Rare Report

Otorhinolaryngology Clinics - An International Journal ◽

10.5005/aijoc-7-1-16 ◽

2015 ◽

Vol 7 (1) ◽

pp. 16-18

Author(s):

Pratik Dipak Shah ◽

Srijon Mukherji

Keyword(s):

Langerhans Cell Histiocytosis ◽

Eosinophilic Granuloma ◽

Maxillofacial Surgery ◽

Langerhans Cell ◽

The Body ◽

Cortical Plate ◽

Dramatic Improvement ◽

Oral Manifestation ◽

Immature Dendritic Cells ◽

Long Term Follow Up

ABSTRACT Introduction Langerhans cell histiocytosis (LCH) is a relatively rare and unique benign disease characterized by an abnormal proliferation of immature dendritic cells which usually affects children and young adults. Jaws are involved in less than 10% of children with the disease. Objective To add on to the literature one more case of this rare and unique disease which accounts for less than 1% of all bone tumors and also to discuss its oral manifestation and management. Design Case report Setting Private maxillofacial surgery institute, Kolkata, West Bengal. Patient A 2 years and 8 months old male child with solitary swelling over the lower left jaw since one and half months. Lesion was fixed to underlying bone and there was an expansion of buccal cortical plate at the body of mandible. Results Enucleation of the lesion performed after assessing history, clinical and radiological dertails and also considering patient's age. Postoperative biopsy result came as LCH presenting as a unifocal eosinophil granuloma. Appropriate surgical intervention resulted in a dramatic improvement in patient's clinical condition. At present, patient is disease-free and asymptomatic. Conclusion Surgical enucleation or curettage is a preferred treatment option for maxillary and mandibular lesion. Potential for the unifocal disease to become multifocal should not be underestimated as the disease can be unpredictable. We recommend long-term follow-up due to uncertainty of the disease.

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Langerhan’s cell histiocytosis with oral manifestation in a 3-year-old child: a case report

Research Society and Development ◽

10.33448/rsd-v10i5.15136 ◽

2021 ◽

Vol 10 (5) ◽

pp. e40310515136

Author(s):

Joana Leticia Vendruscolo ◽

Bruna da Fonseca Wastner ◽

Cleverson Patussi ◽

Sergio Ossamu Ioshii ◽

Juliana Lucena Schussel ◽

...

Keyword(s):

Langerhans Cell Histiocytosis ◽

Soft Tissues ◽

Osteolytic Lesion ◽

Immunohistochemical Analysis ◽

Langerhans Cell ◽

Submandibular Region ◽

Oral Manifestation ◽

S 100 ◽

The Right ◽

Single Lesion

Langerhans cell histiocytosis (LCH) is a disorder that may affect the bones, skin, liver, lung, and hematopoietic and neuroendocrine systems. This condition may manifest as a single lesion, multiple lesions, or as a disseminated and potentially fatal disease. We aim to report a case of a 3-year-old child with LCH in the mandible, sharing with the readers the challenging process of this diagnosis. A three-year-old male patient with persistent swelling in the right submandibular region was referred to the Department of Pediatrics of the Erasto Gaertner Hospital for an evaluation. Initial physical exam revealed a diffuse flaccid swelling occupying the entire right mandibular ramus, from the angle to the preauricular region and CT scan showed an osteolytic lesion with erosion of the internal and external cortices of the mandible and an extension to soft tissues that displaced the masseter muscle. Immunohistochemical analysis confirmed the diagnosis of Langerhans cell histiocytosis through positive tests for CD1a, CD68, S-100, and Vimentin. The treatment proposed was a combination of Vinblastine 6 mg/m3 for 6 weeks and Prednisone 40mg for 4 weeks.The differential diagnosis included pathologies such as rhabdomyosarcoma, Ewing's sarcoma, and, less likely, osteosarcoma and central giant cell granuloma.

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Langerhans Cell Histiocytosis Presenting as a Unifocal Eosinophilic Granuloma in the Body of the Mandible: A Rare Report

Otorhinolaryngology Clinics - An International Journal ◽

10.5005/jp-journals-10003-1158 ◽

2014 ◽

Vol 6 (2) ◽

pp. 74-76

Author(s):

Pratik Dipak Shah ◽

Srijon Mukherji

Keyword(s):

Langerhans Cell Histiocytosis ◽

Eosinophilic Granuloma ◽

Maxillofacial Surgery ◽

Langerhans Cell ◽

The Body ◽

Cortical Plate ◽

Dramatic Improvement ◽

Oral Manifestation ◽

Immature Dendritic Cells ◽

Rare Report

ABSTRACT Introduction Langerhans cell histiocytosis (LCH) is a relatively rare and unique benign disease characterized by an abnormal proliferation of immature dendritic cells which usually affects children and young adults. Jaws are involved in less than 10% of children with the disease. Objective To add on to the literature one more case of this rare and unique disease which accounts for less than 1% of all bone tumors and also to discuss its oral manifestation and management. Setting Private maxillofacial surgery institute, Kolkata, West Bengal. Patient A 2 years and 8 months old male child with solitary swelling over the lower left jaw since one and half months. Lesion was fixed to underlying bone and there was an expansion of buccal cortical plate at the body of mandible. Results Enucleation of the lesion performed after assessing history, clinical and radiological dertails and also considering patient's age. Postoperative biopsy result came as LCH presenting as a unifocal eosinophil granuloma. Appropriate surgical intervention resulted in a dramatic improvement in patient's clinical condition. At present, patient is disease-free and asymptomatic. Conclusion Surgical enucleation or curettage is a preferred treatment option for maxillary and mandibular lesion. Potential for the unifocal disease to become multifocal should not be underestimated as the disease can be unpredictable. We recommend long-term follow-up due to uncertainty of the disease. How to cite this article Shah PD, Mukherji S. Langerhans Cell Histiocytosis Presenting as a Unifocal Eosinophilic Granuloma in the Body of the Mandible: A Rare Report. Int J Otorhinolaryngol Clin 2014;6(2):74-76.

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