J Korean Assoc Maxillofac Plast Reconstr Surg.  2008 Nov;30(6):577-583.

Langerhans Cell Histiocytosis in the Juvenile Mandible

• Kang YH
• Park SH
• Seo DJ
• Cha IH
• Yi CK
• Kim HS
• Kim J
• Kim HJ

Abstract

Langerhans cell histiocytosis (LCH) is characterized by proliferation of pathological Langerhans cells within different organs. It mainly affects children, but adult cases also occur, with an incidence rate of one to two per million.1,2) LCH results from the clonal proliferation of Langerhans cells. And its etiopathogenesis is still unknown.2,3) The hypothesis that it is a neoplastic or inflammatory disease, as well as the existence or not of immunological, viral or genetic predisposing factors, has been widely discussed in the literature, but no conclusive proof has ever been provided.4) Although lesions may appear in tissues of various origins such as skin, hypothalamus, liver, lung, or lymphoid tissue, bone is the most common site of the disease. The head and neck are affected in almost 90% of cases. The maxillary and mandibular bones are affected in 5 to 10% of cases.1,5) In our report, we present four cases of LCH in patients aged 3, 4, 7 and 9 years respectively, with primary manifestation in maxillofacial area.

Keyword

Langerhans cell histiocytosis; Histiocytosis-X; Hand-Schuller-Christian disease; Eosinophilic granuloma; Letterer-Siwe disease

MeSH Terms

Adult
Aged
Child
Eosinophilic Granuloma
Head
Histiocytosis, Langerhans-Cell
Humans
Hypothalamus
Incidence
Langerhans Cells
Liver
Lung
Lymphoid Tissue
Mandible
Neck
Skin