Nasal oral fistula revealing Langerhans' cell histiocytosis in adult patient: case report

Abstract

Langerhans cell histiocytosis (LCH) is a rare systemic disease caused by proliferation of mature histiocytes; its association to histiocyto fibroma is rarely reported. It rarely affects adults. We report a case of systemic LCH, in an adult patient with osteolytic lesion causing a fistula between the left nasal cavity and hard palate, involving the bone, lung, lymph node and associated to multiple histiocyto fibroma. The patient was operating for a fistula, and he was treated by chemotherapy and corticosteroids. Langerhans´ cell histiocytosis is a rare case, especially in adult patient. The diagnosis was based on histological and immunohistochemical analyses. This patient was treated by steroids and chemotherapy.

Langerhans Cell Histiocytosis in the Nasal Bone: A Rare Case

“…Although LCH involving bony structures is more common, LCH can involve any organs despite single site involvement being more common than multi-site involvement ( 3 4 ). Although LCH has been reported to affect the head and neck, involvement of the nasal bone is rare ( 6 7 ).…”

“…LCH predominantly affects children aged 5–15 years ( 3 4 ) and is more prevalent in males than females (M:F; 2:1) ( 3 5 6 ). Although its etiology remains unknown, uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid dendritic cells are presumed to play a role ( 1 6 7 ).…”