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Case Reports
. 2018 Jun 8;18(1):106.
doi: 10.1186/s12903-018-0568-5.

Oral manifestation of Langerhans cell histiocytosis: a case report

Julia Luz  1 , Daniel Zweifel  2 , Martin Hüllner  3 , Marco Bühler  4 , Martin Rücker  2 , Bernd Stadlinger  2
Affiliations
Case Reports

Oral manifestation of Langerhans cell histiocytosis: a case report

Julia Luz et al. BMC Oral Health. .

Abstract

Background: Bone necrosis of the jaw is a serious condition with a broad differential diagnosis of pathologies such as cutaneous histiocytosis, bone metastases or malignant tumours. In addition to the most common cause, medication related osteonecrosis of the jaw (MRONJ), one must consider a number of other causes, such as histiocytosis. Langerhans cell histiocytosis (LCH) is a histiocytic disorder with a large spectrum of clinical manifestations and with possible involvement of a variety of organs. This case shows the importance of an early detection of this rare disease in order to prevent further spreading. Even if an initial diagnosis in the oral cavity is rare, dentists should be aware of this disease.

Case presentation: The presented case describes a patient who was referred for evaluation and treatment due to exposed bone and extensive osteolysis in the region of the upper and lower jaw. After biopsy and diagnosis of LCH, the patient was treated with systemic therapy, achieved remission and is disease free after a 2 year of follow up.

Conclusions: This case report illustrates that when dealing with unclear osteolytic changes of the jawbone, Langerhans cell histiocytosis must be taken into consideration in the differential diagnosis and biopsy must be performed in case of suspicion.

Keywords: Eosinophilic granuloma; Histiocytosis; Langerhans-cell; Osteonecrosis.

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Conflict of interest statement

Ethics approval and consent to participate

Not applicable.

Consent for publication

The patient signed an approval for the publication of a case report under anonymized conditions.

Competing interests

The authors declare that they have no competing interests.

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Figures

Fig. 1
Fig. 1
Clinical presentation of dehiscent gingiva in the mandible (a) and maxilla (b)
Fig. 2
Fig. 2
Baseline FDG-PET/CT scan shows lytic lesions in the right mandible (a, arrow) and left maxilla (b, arrow) with intense radiotracer uptake
Fig. 3
Fig. 3
Coronal PET image in maximum intensity projection shows the FDG-avid lysis in the mandible on the right side and in the maxilla on the left side, as well as other FDG-avid lytic lesions in the right-sided mastoid process as well as in the right-sided skull base
Fig. 4
Fig. 4
The baseline CBCT shows bone resorption in the right mandible and the left maxilla in a reconstructed panoramic view (a) and bone resorption in the right mandible in a 3D-reconstruction (b)
Fig. 5
Fig. 5
Photomicrographs show histiocytic infiltrates (a) with marked eosinophilia (b) (Hematoxylin and Eosin stain)
Fig. 6
Fig. 6
Immunohistochemistry shows the typical LCH phenotype CD1a (a), Langerin (b) and S-100 (c)
Fig. 7
Fig. 7
Clinical presentation of right crestal mandibular (a) and left crestal maxillary (b) gingiva at follow-up after eight months of chemotherapy
Fig. 8
Fig. 8
The follow-up CBCT shows bone regeneration in the right mandible and the left maxilla in a reconstructed panoramic view (a) and bone regeneration in the right mandible in a 3D-reconstruction (b)
Fig. 9
Fig. 9
Follow-up FDG-PET/CT shows absence of pathologic radiotracer uptake within the lytic lesions in the right mandible (a, arrow) and the left maxilla (b, arrow)
See this image and copyright information in PMC

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