Oral manifestation of Langerhans cell histiocytosis: a case report

Abstract

BackgroundBone 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 presentationThe 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.ConclusionsThis 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.

Langerhans cell histiocytosis: A case report with oral manifestations and the role of pediatric dentists in the diagnosis

“…Oral manifestations may include gingivitis, periodontitis, tooth rotation or loss, and malocclusion. In the present case, the osteolytic lesions were encountered in the maxillary and mandibular bones causing hypermobility of the teeth (floating teeth) …”

“…FDG‐PET scan is required to exclude intense radiotracer activity in other sites of the body and to assess the response for treatment; in the present case, Tc 99m ‐MDP Bone Isotope scan was used and showed increased radiotracer uptake within the maxillary and mandibular bones only . A previous molecular study showed a six base pair deletion in exon 3 of the MAP2K1 gene (p.E102_I103del) . A genetic study was not ordered in this case.…”

“…Although the disease is usually encountered in patients between 1 and 10 years of age with male predominance, recently reported cases described LCH in a five‐month‐old infant and patients aged between 17 and 63 years old. The present article discusses the case of a 2‐year‐old male …”

“…Recurrent mouth ulceration and gingival necrosis are nonspecific signs that can be caused by various disorders such as leukocyte adhesion deficiency (LAD), hypophosphatasia, scurvy, leukemia, and medication‐related osteonecrosis of the jaw, but when there is hypermobility of teeth, then bone destruction should be suspected and radiological investigation should be requested and this emphasizes the rule of a pediatric dentist in diagnosis of systemic disorders such as LCH . The definitive diagnosis of LCH is made by histopathology where sheets of large histiocytosis with longitudinal groove (coffee‐bean‐like) nuclei and typical and atypical mitoses in a eosinophils rich background, and immunohistochemical stains are of utmost importance in highlighting reactivity to S‐100 and CD1a . A monoclonal antibody against CD207 (langerin) is a highly specific and sensitive protein that is needed for formation of the Birbeck‐Broadbent granules .…”

“…Because of the clinical variability of the disease and lack of standard diagnostic tools and evaluation, optimal treatment of LCH is still controversial and depends on the age of the patient and the extent of the disease. Small solitary osteolytic lesions or single system disease exhibiting benign course is usually treated with surgical curettage followed by a local injection of steroids for the remission of symptoms . However, radiotherapy or chemotherapy, or both are needed in case of multiple organ involvement, a big solitary lesion, or recurrence .…”