Solitary langerhans cell histiocytosis in an adult: case report and literature review

Abstract

BackgroundLangerhans cell histiocytosis (LCH) is a disease that often affects children, but can also occur in adults and smokers. Oral manifestations are unusual and are characterized by bone pain, tooth mobility, necrotic ulcers and local edema. The aim of this paper is to describe a clinical case of LCH in an oral cavity that mimicked oral squamous cell carcinoma.Case presentationA male, 63 years old, complaining about a “wound in the mouth” for 6 months, without any pain or spontaneous bleeding. His medical history was free of disease. The patient was a smoker for 33 years. Intraoral examination revealed a destructive ulcerative lesion around the upper left first and second molars that resembled an oral squamous cell carcinoma. Biopsy of the ulcerative lesion was performed and the microscopic features showed an inflammatory infiltrate rich in plasma cells. Based on this microscopical finding, the final diagnosis was periodontal disease associated with a proliferative non-neoplastic lesion. The patient was referred to a specialized dental surgeon and underwent periodontal therapy including surgical procedures. After that, according to follow-up with the patient, there were no signs of disease remission. The lesion increased in size, although the patient did not complain of any symptoms. A second biopsy was performed and the microscopic features again showed a rich inflammatory infiltrate with mononuclear cells and histiocytic cells, characterized by pale histiocytes with lobed nuclei, resembling a bean. A varying number of eosinophils also were observed, without any evidence of atypical cells present in this infiltrate. An immunohistochemical staining panel was done to determine the nature of this inflammatory infiltrate by using antibodies S-100, CD1a, CD-68 and CD45RO that were positive. These immunohistochemical findings were fundamental for the final diagnosis of LCH. The treatment included surgical extraction of all superior teeth, radiation and systemic corticoid therapies. After 8 years of treatment, the patient is free of disease.ConclusionAlthough LCH is an unusual lesion in an oral cavity, it can be present. Biopsy and a histological exam are essential to establish the diagnosis. Immunohistochemicals were fundamental to exclude malignant lesion and to confirm the diagnosis of LCH.

Langerhans Cell Histiocytosis Mimicking Aggressive Periodontitis. A Multidisciplinary Approach During an 8‐Year Follow‐Up Period

“…In the case of LCH, systemic treatment is crucial for the arrest of periodontal disease. In agreement with previous reports and reviews of the literature, 4‐15 the present case suggests that periodontal lesions can be diagnostic criteria for LCH. The present report also demonstrates the need for a concise and strict multidisciplinary approach for these patients, even when they are not compliant with therapy.…”

“…The clinical presentation is heterogeneous, and the patient may be asymptomatic, especially in cases of solitary, osteolytic lesions, or have severe symptoms, in the systemic form of the disease. In both cases, LCH lesions may be present in any tissue or organ, involving the oral cavity and periodontal tissues 3‐15 . Diagnosis of LCH requires a tissue biopsy and the demonstration of CD1α antigenic determinants on the surface of lesional cells 3 .…”

“…In both cases, LCH lesions may be present in any tissue or organ, involving the oral cavity and periodontal tissues. [3][4][5][6][7][8][9][10][11][12][13][14][15] Diagnosis of LCH requires a tissue biopsy and the demonstration of CD1a antigenic determinants on the surface of lesional cells. 3 To date, there is no standard first-line therapy for LCH in adults, in contrast with the established protocols in children.…”

Langerhans cell histiocytosis of a metatarsal bone in an adult female

“…Treatment modalities may range from (1) surgical procedures, like curettage or excision, (2) irradiation, (3) intralesional steroid injection and (4) chemotherapy. The choice of treatment should be based on the pattern of involvement, that is, single or multisystem involvement and also the size, site of the lesion and age of the patient 16. Curettage is best for easily assessable unifocal small size lesions,17 as was done in our case.…”

Spontaneous remission of eosinophilic granuloma of the maxilla after incisional biopsy: a case report

“…A key feature of this neoplasm is its clonal derivation from a single cell, which does not necessarily mean malignancy [ 6 ]. On the other hand, it is reported that proinflammatory cytokines and chemokines as well as oncogenic B-type Raf kinase (BRAF) gene mutations are involved in the pathogenesis of EG [ 10 ]. BRAF mutations are present in several malignant neoplasias including papillary thyrd carcinoma [ 11 ].…”