Bilateral synchronous presentation of Langerhans cell histiocytosis of maxilla and mandible: a rare case report and literature review
DOI:
https://doi.org/10.18203/2349-2902.isj20261191Keywords:
Langerhans cell histiocytosis, Immunohistochemistry test, Antigen presenting cellsAbstract
Previously known as histiocytosis X, Langerhans cell histiocytosis (LCH) is a rare haematological condition that primarily affects new-borns and young children. The uncontrolled activation and proliferation of normal antigen-presenting cells, specifically Langerhans cells, are the defining features of this condition. A positive immunohistochemistry result for CD1a/CD207 and S100 is the gold standard for a conclusive diagnosis. There are various therapy options available for individuals with LCH. Due to its comparatively low occurrence, little information is known about the epidemiology of LCH; cases are estimated to occur 3-5 times annually per million population. In this report, we have highlighted a case of 35-year-old adult who reported to the department with Bilateral synchronous gingival enlargements in maxilla and mandible. On clinical, radiological, histopathological (Incisional biopsy) and immunohistochemical examinations revealed a diagnosis of LCH. even though LCH incidence in adults is uncommon, LCH must be taken into account when evaluating multiple osteolytic bony jaw lesions in young adults that have an unclear aetiology. So here we present a rare case of Bilateral synchronous presentation of LCH of maxilla and mandible in adult with its management.
References
Bhargava D, Bhargava K, Hazarey V, Ganvir SM. Hand-Schüller-Christian disease. Indian J Dental Res. 2012;23(6):830-2.
Scolozzi P, Lombardi T, Monnier P, Jaques B. Multisystem Langerhans’ cell histiocytosis (Hand-Schüller-Christian disease) in an adult: a case report and review of the literature. Europ Arch Oto-Rhino-Laryngol Head Neck. 2004;261:326-30.
DM Howarth. Langerhans cell histiocytosis diagnosis, natural history, management, and outcome. Cancer. 1999;85:2278-90.
Allen CE, Merad M, McClain KL. Langerhans-cell histiocytosis. N Eng J Med. 2018;379(9):856-68.
Stockschlaeder M, Sucker C. Adult Langerhans cell histiocytosis. Europ J Haematol. 2006;76(5):363-8.
Lian C, Lu Y, Shen S. Langerhans cell histiocytosis in adults: a case report and review of the literature. Oncotarget. 2016;7(14):18678.
Madrigal-Martínez-Pereda C, Guerrero-Rodríguez V, GuisadoMoya B, Meniz-García C. Langerhans cell histiocytosis: literature review and descriptive analysis of oral manifestations. Med Oral Patol Oral Cir Bucal. 2009;14:222-8.
Chu T. Langerhans cell histiocytosis. Aust J Dermatol. 2001;42(4):237-42.
Campanati A, Simonetti O, Marconi B, Giuliodori K, Ganzetti G, Brandozzi G, et al. Purely cutaneous Langerhans’ cell histiocytosis in an adult woman. Acta Derm Venereol. 2009;89(3):299-301.
Zhinan Zhang, Ti Shen. Diagnostic and curative standard of blood disease. Edition 3. Beijing: Science Publishing Company. 2007;7:248-53.
Neves-Silva R, Fernandes DT, Fonseca FP, Rebelo Pontes HA, Brasileiro BF, Santos-Silva AR, et al. Oral manifestations of Langerhans cell histiocytosis: A case series. Spec Care Dentist. 2018;38(6):426-33.
Hicks J, Flaitz CM. Langerhans cell histiocytosis: current insights in a molecular age with emphasis on clinical oral and maxillofacial pathology practice. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005;100:S42-66.
Annibali S, Cristalli MP, Solidani M, Ciavarella D. Langerhans cell histiocytosis: oral/periodontal involvement in adult patients. Oral Dis. 2009;15:596-601.
Eckardt A, Schultze A. Maxillofacial manifestations of Langerhans cell histiocytosis: a clinical and therapeutic analysis of 10 patients. Oral Oncol. 2003;39:687-94.
Bartnick A, Friedrich RE, Roeser K, Schmelzle R. Oral Langerhans cell histiocytosis. J Craniomaxillofac Surg. 2002;30:91-6.
Hartman KS. Histiocytosis X: a review of 114 cases with oral involvement. Oral Surg Oral Med Oral Pathol. 1980;49:38-54.
Rao DG, Trivedi MV, Havale R, Shrutha SP. A rare and unusual case report of Langerhans cell histiocytosis. J Oral Maxillofac Pathol. 2017;21(1):140-4.
Baptista AM, Camargo AF, de Camargo OP, Odone Filho V, Cassone AE. Does adjunctive chemotherapy reduce remission rates compared to cortisone alone in unifocal or multifocal histiocytosis of bone. Clin Orthop Relat Res. 2012;470:663-9.
Quraishi MS, Blayney AW, Breatnach F. Aural symptoms as primary presentation of Langerhan's cell histiocytosis. Clin Otolaryngol Allied Sci. 1993;18:317-23.