Langerhans cell histiocytosis (LCH) in orofacial region


Authors: A. Fassmann 1;  L. Izakovičová Hollá 1, 2;  P. Augustín 1;  J. Vokurka 1;  J. Vaněk 1
Authors‘ workplace: Stomatologická klinika Lékařské fakulty MU a FN u sv. Anny Brno, přednosta prof. MUDr. Jiří Vaněk, CSc. 1;  Ústav patologické fyziologie Lékařské fakulty MU Brno, přednostka prof. MUDr. Anna Vašků, CSc. 2
Published in: Vnitř Lék 2010; 56(Supplementum 2): 85-90
Category: Langerhans cell histiocytosis and some other Hematology rare diseases

Overview

Langerhans cell histiocytosis (LCH) is a rare disease of unknown origin. It is characterized by intense and abnormal proliferation of bone marrow-derived histiocytes (Langerhans cells). This disease has a broad spectrum of forms and severity of clinical manifestations, it invades bones, skin, mucosa as well as the inner organs. This case report describes a 33-year monitoring of a patient with a chronic diffuse form of LCH, so called Hand-Schüller-Christian disease. Apart from skin manifestations, this patient also had infiltrates of the oral mucosa, and osteolytic lesions of the alveolar bone of both upper and lower jaws. These findings helped dermatologists confirm the diagnosis of LCH. The dentist plays an important role in multidisciplinary treatment of patients with LCH as periodic checkups with emphasis on possible oral manifestations can reveal the first signs of LCH.

Key words:
Langerhans cell histiocytosis – histiocytosis X – oral manifestations – infiltrate – bone lesion


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Diabetology Endocrinology Internal medicine

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