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ORIGINAL ARTICLE Oral Manifestations May Be the First Sign of Langerhans Cell Histiocytosis Arzu Pinar Erdema/Yelda Kas...

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ORIGINAL ARTICLE

Oral Manifestations May Be the First Sign of Langerhans Cell Histiocytosis Arzu Pinar Erdema/Yelda Kasimoglub/Elif Sepetc/Koray Gencayc/Songul Sahind/ Sergulen Dervisoglue Abstract: Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of histiocytes characterised by a proliferation of abnormal and clonal Langerhans cells. LCH most commonly occurs as a localised solitary bone lesion and appears predominantly in paediatric patients. This case resport presents clinicopathological features of this disorder in a 2-year-old boy. The presenting features of the patient included fever, bleeding gums and seborrheic dermatitis-like rash. Intraoral examination and radiographs revealed deep pockets and severe bone loss around all primary molars. Due to severe periodontal destruction, a gingival biopsy was taken. The biopsy specimen revealed heavy infiltration of Langerhans histiocytes. Chemotherapy was administered. Although no clear cause of LCH has been identified, the disease can be triggered by environmental agents and viruses, in particular Epstein-Barr virus. Oral manifestations may be the first or only signs of LCH. Key words: Langerhans cell histiocytosis, periodontal disease Oral Health Prev Dent 2013; 11: 57-59

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angerhans cell histiocytosis (LCH) is a rare disease of unknown pathogenesis, characterised by intense and abnormal proliferation of bone marrowderived histiocytes (Langerhans cells). It can present both local and systemic manifestations involving bone, skin, mucosal tissue and internal organs. Three basic clinical forms develop: Letterer-Siwe disease (subacute or acute disseminated form), Hand-Schüller-Christian disease (disseminated chronic form) and eosinophilic granuloma (localised chronic form). LCH may manifest orally with single or multiple lesions of the alveolar or basal bone, ulcerated mucosal lesions accompanied by adenopathies and/or periodontal lesions, present-

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Research Assistant Doctor, Department of Paedodontics, Faculty of Dentistry, Istanbul University, Capa, Istanbul, Turkey.

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Research Assistant, Department of Paedodontics, Faculty of Dentistry, Istanbul University, Capa, Istanbul, Turkey.

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Professor, Department of Paedodontics, Faculty of Dentistry, Capa, Istanbul University, Istanbul, Turkey.

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Medical Doctor, Department of Pathology, Cerrahpaüa Faculty of Medicine, Istanbul University, Istanbul, Turkey.

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Professor, Department of Pathology, Cerrahpaüa Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Correspondence: Dr. Arzu Pinar Erdem, Department of Paedodontics, Faculty of Dentistry, Istanbul University, Capa, 34093 Istanbul, Turkey. Tel:+90-212-414-2020/30309, Fax: +90-212-531-0515. Email: [email protected]

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Submitted for publication: 21.01.12; accepted for publication: 30.04.12

ing gingival inflammation, bleeding, recession, necrosis, odontalgia, dental hypermobility and premature loss of teeth. The principal differential diagnosis includes advanced periodontal disease or a periapical process of dental or periodontal origin. The dentist plays a vital role in the diagnosis and multidisciplinary treatment of such patients, by performing routine examinations for periodic follow-up of the disease and its possible oral manifestations, bearing in mind that these may be the first or only signs of LCH (Hernandez-Juyol et al, 2003; Madrigal-Martinez-Pereda et al, 2009). The disease has an incidence of 1 per 1,000,000 in children younger than 15 years, with a peak incidence between the ages of 1 and 4 years. The pathogenesis of LCH is unknown (Punia et al, 2006).

CASE REPORT A 2-year-old boy was referred to the Department of Paedodontics, Istanbul University by his general dental practitioner. The presenting features of the patient included fever, bleeding of gums around his newly erupting deciduous molars and seborrheic dermatitis-like rash.The lesions were observed in abdominal and head regions and were 1–2 mm in

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Fig 1  The radiographic examination of the patient revealed deep pockets and severe bone loss around all primary molars.

Fig 2  The intraoral examination revealed markedly reddened and swollen gingiva around mandibular primary molars.

Fig 3  Gingival tissue revealing heavy infiltration of Langerhans histiocytes beneath surface epithelium. These are immunoreactive with CD1a and langerin.

diameter. The medical history revealed fever and clinical and laboratory evidence of Epstein-Barr virus infection at 16 months of age. Intraoral examination and radiographs revealed deep pockets and severe bone loss around all primary molars (Fig 1). The gingiva was markedly reddened and swollen around the mandibular primary molars (Fig 2). Further haematological and immunological investigations were carried out. The results were essentially normal. Due to severe periodontal destruction, a gingival biopsy was taken, which revealed heavy infiltration

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of Langerhans histiocytes. Immunohistochemical staining with antibodies to CD1a, Langerin (CD207) and S100 was strongly positive (Fig 3). Based on these results, the diagnosis of Langerhans cell histiocytosis was made. Chemotherapy was administered.

DISCUSSION Langerhans cell histiocytosis (LCH) is a rare disorder characterised by an abnormal accumulation

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and/or proliferation of cells with a Langerhans cell phenotype. Although no clear cause of LCH has been identified, it has been postulated that LCH might be the consequence of an immune dysregulation, causing Langerhans cells to migrate to and accumulate at various sites (Madrigal-MartinezPereda et al, 2009). The role of several human herpes viruses (HHV) in the pathogenesis of LCH has been suggested by numerous reports but remains controversial. Epstein-barr virus (EBV, HHV-4) and cytomegalovirus (CMV, HHV-5) can infect Langerhans cells, and EBV, CMV and HHV-6 have been proposed to be associated with LCH based on the detection of these viruses in clinical samples (Sakata et al, 2008; Minkov, 2011). Patients with multisystem LCH represent a heterogeneous group with respect to disease severity and outcome; therefore, treatment stratification and risk-tailored treatment are mandatory. The risk for mortality can be predicted based on the involvement of risk organs (e.g. hematopoietic system, liver and /or spleen) at diagnosis and on response to initial therapy (assessed after 6 to 12 weeks of treatment). Thus, patients without involvement of risk organs (low-risk group) are not at risk for mortality but still need systemic therapy in order to control the disease activity and avoid reactivations and permanent consequences (Minkov, 2011). This case report dealt with a 2-year-old male patient infected with Epstein-Barr virus at 16 months of age, who subsequently developed Langerhans cell histiocytosis. The patient belongs to a low-risk group without involvement of risk organs. He is not at mortal risk, but needs systemic therapy. In cases of LCH with oral manifestations, it is not necessary to extract all the teeth involved in the process, only those with marked mobility, or with periapical lytic lesions and those presenting symptoms. Correct periodontal treatment and rigorous hygiene must be maintained to conserve both teeth and periodontal tissue.

The dentist plays a vital role in the diagnosis and multidisciplinary treatment of such patients by performing routine examinations for periodic follow-up of the disease and its possible oral manifestations, bearing in mind that these may be the first or only signs of LCH.

ACKNOWLEDGEMENTS We thank to Professor Dr. Gulsum Ak from Istanbul University, Faculty of Dentistry, Department of Oral Medicine and Surgery, Capa, Istanbul, Turkey for her special interest and help in this case.

REFERENCES 1. Hernandez-Juyol M, Boj-Quesada JR, Gallego Melcon S. Oral manifestations of Langerhans cell histiocytosis. Case study of a two-year-old boy. Medicina Oral, Patologia Oral, Cirugia Bucal 2003;8:19-25. 2. Madrigal-Martinez-Pereda C, Guerrero-Rodriguez V, GuisadoMoya B, Meniz-Garcia C. Langerhans cell histiocytosis: literature review and descriptive analysis of oral manifestations. Medicina Oral, Patologia Oral, Cirugia Bucal 2009;14:222-228. 3. Minkov M. Multisystem Langerhans cell histiocytosis in children: current treatment and future directions. Paediatric Drugs 2011;13:75-86. 4. Punia RS, Bagai M, Mohan H, Thami GP. Langerhans cell histiocytosis of skin: A clinicopathologicanalysis of five cases. Indian Association of Dermatologists, Venereologists & Leprologists 2006;72:211-214. 5. Sakata N, Toguchi N, Kimura M, et al. Development of Langerhans cell histiocytosis associated with chronic active Epstein-Barr virus infection. Pediatric Blood & Cancer 2008;50:924–927.

CONCLUSION Alveolar bone loss in young children is clearly a serious finding. This disease is of clinical importance to dental professionals because LCH commonly involves the oral and maxillofacial region and early symptoms can be manifested in the jaw. In children who present features of prepubertal periodontitis, the differential diagnosis should always include LCH.

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