Langerhans Cell Disease: Langerhans Cell Histiocytosis
Etiology: The basic cause of Langerhans cell disease, formally called histiocytosis X, is unknown. It involves proliferation of the Langerhans cells, which normally reside in the epidermis, bone marrow, lymph nodes, and mucosa. Langerhans cells are a specialized histiocyte involved in antigen processing for our immune system. They have unique Birbeck granules in their cytoplasm ultrastructurally. The Langerhans are usually mixed with eosinophils histologically. In some cases, a neoplastic transformation is believed responsible for this disease. An immune response has also been implicated. Additionally, a neoplastic process and a reactive process have been suggested as causal.
Mode of transmission: NA
Characteristics: Poor healing is common with Langerhans cell disease, dermatologic conditions may be evident, and all bones of the body may be affected. Lymphadenopathy may be present, cranial bones may be involved, and the cutaneous areas may involve rashes and erythematic lesions. Radiographically, the bone lesions resemble a “punched-out” appearance with sharply demarcated lesions. Lesions commonly present as a “floating tooth/teeth.” The disease may involve one or more multiple bones in the body, including the bones around the teeth, contributing to loosening (called “floating teeth”). Tenderness, pain, and swelling are common complaints. The patients may overlap in the above stated symptoms and may or may not exhibit the stated characteristic symptoms, depending upon the stage and type of the disorder.
Implications: Lesions that occur periapically can be confused with periapical cyst or granulomas. Tooth vitality would still be present.
Treatment: The treatment depends upon the involvement of the disease and the age of the patient. Conservative surgical treatment is sometimes the only treatment. However, in more extensive disease involvement, chemotherapy may be needed as well. Additionally, bone marrow transplantation may be done; but essentially, the more widespread the lesions, the poorer the prognosis. The prognosis is more favorable when the disease states develop in older young adults.