Histiocytosis is a generic name for a group of syndromes varcharacterized by an abnormal increase in the number of certain immune cells called histiocyte cells. These include monocytes, macrophages, and dendritic cells This syndrome has been known under a variety of names including: Eosinophilic Granuloma, Hand-Schuller-Christian Disease, Langerhans Cell Histiocytosis, Letterer-Siwe Disease, Nonlipid Reticuloendotheliosis, Pulmonary Histiocytosis X, Pulmonary Langerhans Cell Granulomatosis. There are three major classes of Histiocytosis. These include Histiocytosis X, hemophagocytic syndrome and malignant Histiocytosis syndrome (now known as T-cell lymphoma). Most cases of Histiocytosis X affect children between ages 1 and 15 years old. The incidence peaks amongst children between 5 and 10. Histiocytosis X is thought to affect roughly 1 in 200,000 people each year. The extra immune cells may form tumors, which can affect various parts of the body. In children, Histiocytosis X usually involves the bones (80%) and may consist of single or multiple sites. The skull is frequently affected.
Causes of Schuller-christian Syndrome
Historically, Hand-Schuller-Christian disease has been thought of as a cancer like condition. More recently, however, researchers have begun to suspect that it is actually an autoimmune phenomenon, in which rogue immune cells attack the body, rather than fighting infections. Some forms of this syndrome are genetic. Pulmonary Histiocytosis X is varcharacterized by inflammation of the small airways (bronchioles) and the small blood vessels in the lungs. It is most common in adults. The inflammation leads to stiffening (fibrosis) and destruction of the walls of the alveoli (parts of the lung). The cause is unknown. People 30 to 40 years old are affected most often. 90% of patients are cigarette smokers
Signs and Symptoms of Schuller-christian Syndrome
Symptoms or Hand-Schuller-Christian disease are frequently the result of abnormal deposits of Histiocyte. These deposits lead to weakened bones. When the weight bearing bones, such as the legs or spine are involved, they may fracture spontaneously or after minimal trauma. There is often systemic involvement that can lead to generalized symptoms and ill feeling. Not surprisingly, with a disease that affects so many systems and organs, Histiocytosis may be deadly. Adults may develop cough, shortness of breath and chest pain. Fever, weight loss and rash may occur. Due to involvement of the pituitary gland, hormones that regulate urine output may become affected. This can lead to excessive urination and increased thirst. Pain in one or may bones may occur. Infants may demonstrate a failure to thrive. Development of children can be retarded with short stature and delayed puberty. In addition to the symptoms mentioned above for adults, children may have abdominal pain, vomiting and yellowing of the skin. Headaches, seizures, dizziness and mental deterioration may occur. The eyes may appear swollen or prominent. There may be enlargement of the glands in the neck, armpit or groin. Bone pain may be seen as well as limping.
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Medical Content Last Updated on 07/12/2008
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