Langerhans cell histiocytosis with oral manifestations: a rare and unusual case report

B. Yashoda Devi,N. Rakesh,M. Agarwal

doi:10.4317/jced.50728

Abstract

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. Key words:Langerhans cell histiocytosis, immunohistochemistry, bone scan.

Highlights

Langerhans cell histiocytosis (LCH) is an uncommon disorder of the dendritic Langerhans cell that lacks histologic evidence of malignancy but behaves in an aggressive manner
Etiology is unknown and various theories suggest a role for environmental, infectious, immunologic, genetic causes, and even some believe that LCH is a neoplastic process (3)
There may be severe alveolar bone resorption producing the appearance of teeth ‘‘floating in space’’ (8) as in our case. These non pathognomonic clinical and radiographic features led to the diagnosis of acute nectrotizing ulcerative periodontitis in first place and there was a delay of 90 weeks in diagnosis of LCH

Summary

Introduction

Langerhans cell histiocytosis (LCH) is an uncommon disorder of the dendritic Langerhans cell that lacks histologic evidence of malignancy but behaves in an aggressive manner. We report a case of LCH, which had presented with oral manifestations without any other systemic signs and symptoms. On complete work up the patient was diagnosed to have LCH with systemic involvement. To confirm the lesional Langerhans cells, immunohistochemical staining for CD1a which is diagnostic for it was performed (6) (Fig. 2).

Results

Conclusion