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raz J Otorhinolaryngol. 2014;80(4):366---367

Brazilian Journal of

OTORHINOLARYNGOLOGYwww.bjorl.org

ASE REPORT

ral Langerhans cell histiocytosis: case report withollow-up of ten years�

istiocitose de células de Langerhans oral: relato de caso comcompanhamento de dez anos

meline das Neves de Araújo Lima, Eliakim Medeiros Alves de Araújo,atrícia Teixeira de Oliveira, Ana Miryam Costa de Medeiros ∗

niversidade Federal do Rio Grande do Norte (UFRN), Natal, RN, Brazil

eceived 25 September 2012; accepted 14 March 2013

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vailable online 23 May 2014

ntroduction

angerhans cell histiocytosis (LCH) is characterized by clonalroliferation of Langerhans cells exhibiting Birbeck granulesnd positive immunohistochemistry for S100 and CD1A.1 Aalignant transformation or a functional proliferation of

angerhans cells responding to external stimuli are possibleources.2 In the oral cavity, they can occasionally present asyperplasia of the gingiva or ulcers of the cheek, palate,r tongue mucosa.3 The diagnosis is made after carefulxamination, and the exclusion of other similar diagnosticossibilities.

Several therapeutic modalities have been suggestedor LCH, such as intralesional corticosteroid injection,ntibiotics, steroids, radiation therapy, and chemotherapy.urgical options ranging from extensive resections to moreonservative approaches are available and, in many cases,ealing has resulted from a single biopsy.4

The present report regards LCH in the oral cavity andmphasizes the rarity of this lesion, as well as the impor-

ance of differential diagnosis, treatment, and appropriateollow-up for these patients.

� Please cite this article as: Lima EN, de Araújo EM, de Oliveira PT,e Medeiros AM. Oral Langerhans cell histiocytosis: case report withollow-up of ten years. Braz J Otorhinolaryngol. 2014;80:366---7.∗ Corresponding author.

E-mail: emelinelima@hotmail.com (A.M.C. de Medeiros).

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ttp://dx.doi.org/10.1016/j.bjorl.2014.05.003808-8694/© 2014 Associacão Brasileira de Otorrinolaringologia e Cirueserved.

ase presentation

ten-year-old male with leukoderma presented to thetomatology Clinic, with a three-month history of a lesionn the roof of the mouth. On intraoral examination, aed ulcerated lesion of approximately 1 cm size with anrthodontic ring (Fig. 1A) was seen on the palatal gingivadjacent to the first upper molar. Following periodontalreatment, no improvement was observed, and an initialiagnosis of paracoccidioidomycosis was suggested. Inci-ional biopsy revealed a lesion predominantly consistingf polygonal cells, at times exhibiting granular cytoplasmFig. 1B), consistent with LCH and confirmed by stronglyositive immunohistochemistry for S100 (Fig. 1C) and CD1AFig. 1D). The condition was treated by chemotherapy andurgical removal of the lesion. Over the next ten years, theatient was assessed twice a year, and showed no clinicaligns of relapse. On his last visit, he underwent a bone scannd temporal bone computed tomography and there was novidence of relapse or metastases.

iscussion

he etiopathogenesis of LCH has not been fully determined,

nd a possible reaction or neoplastic phenomena has beenroposed. A few authors further suggest an immune systemegulation disorder and a familial predisposition,5 since itrequently affects children, as in the present report.

rgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights

Oral Langerhans cell histiocytosis 367

Figure 1 (A) Intraoral clinical imaging showing red ulcerated lesion in the palatal gingiva, adjacent to the first upper molar. (B)Microphotograph showing neoplastic lesion characterized by polygonal cells, at times exhibiting granular cytoplasm among numerous

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cytosis in four-month-old child. Int J Pediatr Otorhinolaryngol.2011;75:963---7.

blood vessels, and inflammatory infiltrate consisting of lymphocyical findings positive for S100 (×200). (D) Immunohistochemical

The clinical features of LCH are similar to several con-ditions, including periodontal disease, malignancies such assquamous cell carcinoma or lymphoma, as well as granulo-matous or ulcerative lesions that are characteristic of fungalinfections.2 Thus, a thorough assessment is appropriate fororal cavity lesions that persist after treatment. In the oralcavity, it usually presents as a mucosal ulcer associated withunderlying bone lesions,6 which was not observed in thiscase.

Diagnosis may be confirmed by detecting the charac-teristic Birbeck granules (X bodies) or specific monoclonalantibodies to surface antigens (CD1).6 In the present case,diagnosis was confirmed after an immunohistochemicalstudy that characterized the proliferating cell type.

The therapeutic approach depends on the extent of thedisease and local treatment is usually effective in formslimited to a single organ. However, a few complications,such as pituitary gland malfunction, and especially diabetesand neurodegenerative diseases can occur.5 A retrospectivereview of patients with LCH revealed a high ten-year survivalrate (93%); most individuals are low-risk and have no bonemarrow, spleen, liver, or lung involvement.1 Our patienthas had no relapse or metastasis after a ten-year follow-up; however, long-term follow-up is required to detect andcontrol possible late-onset sequelae.3

Final comments

The present report discussed clinical and laboratory infor-mation on LCH, emphasizing the importance of differential

6

neutrophils, and eosinophils (HE ×400). (C) Immunohistochem-ings positive for CD1A (×400).

iagnosis, appropriate treatment, and long-term follow-upith complication and/or relapse prevention.

onflicts of interest

he authors declare no conflicts of interest.

eferences

. Maria Postini A, Del Prever AB, Pagano M, Rivetti E, Berger M,Asaftei SD, et al. Langerhans cell histiocytosis: 40 years’ experi-ence. J Pediatr Hematol Oncol. 2012;34:353---8.

. Madrigal-Martínez-Pereda C, Guerrero-Rodríguez V, Guisado-Moya B, Meniz-García C. Langerhans cell histiocytosis: literaturereview and descriptive analysis of oral manifestations. Med OralPatol Oral Cir Bucal. 2009;14:222---8.

. Kilic E, Er N, Mavili E, Alkan A, Gunhan O. Oral mucosal involve-ment in Langerhans’ cell histiocytosis: long-term follow-up of arare case. Aust Dent J. 2011;56:433---6.

. Lee S-H, Yoon H-J. Intralesional infiltration of corticosteroids inthe treatment of localized Langerhans cell histiocytosis of themandible: report of two cases. Oral Surg Oral Med Oral PatholOral Radiol. 2012 [in press].

. Martins MAT, Gheno JLN, Sant’Ana Filho M, Pinto Jr DS, TenisCA, Martins MD. Rare case of unifocal Langerhans cell histio-

. Murray M, Dean J, Slater L. Multifocal oral Langerhans cell histi-ocytosis. J Oral Maxillofac Surg. 2011;69:2585---91.