What is histiocytosis of Langerhans cells?
Langerhans cell histiocytosis (LCH) is a rare disease in which Langerhans cells proliferate abnormally. Langerhans cells are part of the immune system, usually found in the skin and lymph nodes. When the cells are produced excessively, they cause damage to the skin, bones and other parts of the body. In the past, histiocytosis of Langerhans cells was referred to as histiocytosis of dendritic cells, histiocytosis X, Hand-Schüller-Christian diseases and the diseases of the letters -iwe. It most often affects children aged five and ten years, with about 200,000 children to ten affected annually and only one of 560,000 adults. Caucasians are more likely to show disease and men are twice as likely to be as women. The unifocal form, sometimes called an outdated term eosinophilic granuloma is a slowly progressive disease in which Langerhans cells are proliferated in one area of the body such as bones, skin, lungs or stomach. MultifocalThe form of the unissystem of the disease causes fever, bone lesions, eruptions on the scalp and in the ears canals and convex eyes. Diabetes insipidus, a condition characterized by excessive thirst and urination, occurs in 50% of the multifocal unisystem LCH. In the multifocal LCH multifocal multisystem, Langerhans cells are rapidly prolonged in many body tissues and the prognosis is very poor, with 50% level of survival for five years.
symptoms of histiocytosis of Langerhans cells depend on the form of disease and affected tissues. Anemia, fever, lethargy and weight loss are common. If bones are affected, symptoms may include painful swelling of bones and lesions. If bone marrow is affected, blood cells may be epicity. Langerhans cell proliferation in the skin causes a rash, leading to lesions and eruptions, especially on the scalp.
If Langerhans cell histiocytosis affects the endocrine glands, diAbetes insipidus and permanent lack of leading pituitary gland are probably symptoms. Lymph nodes, spleen and liver can increase if they are affected by disease. In Langerhans Cell histiocytosis is present in the lungs, the patient may experience shortness of breath or chronic cough.
Langerhans cell histiocytosis is diagnosed with tissue biopsy. Limited, unifocal cases can often be treated with local radiation or surgery and usually have excellent prognosis. Other forms of the disease are usually treated with chemotherapy and steroids. Local steroid creams and medicines can be used for skin lesions or supplements can be solved by endocrine shortages.