Information on Langerhans Cell Histiocytosis

Langerhans cell histiocytosis also known as; Histiocytosis X. Langerhans cell histiocytosis is a rare disorder that arises when there are too lots of of a type of white blood cell called a Langerhans cell. These cells normally reside in the skin and help fight infections and kill certain foreign substances in the body. About 50 children in the UK develop Langerhans' cell histiocytosis each year. It can affect children of any age, and is more common in boys than girls. It is estimated that 8.9 of every 1,000,000 children under the age of 15 have histiocytosis Seventy-six percent of the cases occur before ten (10) years of age.

The disease will be classed as either single-system or multi-system depending on how many of the body's systems is effected. Langerhans cell histiocytosis can also be found in the ribs, sternum, long bones of the arms and legs, vertebra of the spine, and the pelvis. The cause of Langerhans cell histiocytosis is unknown. Viral infections or environmental factors that could lead to this disorder. Cigarette smoking may play a role as a chronic irritant in the development of eosinophilic granuloma of the lung. It is not a known infection or cancer. It is not known to be hereditary or communicable.

It cannot be caught from other people and it is not passed on in families. Symptoms can vary between children and adults, although there can be some overlap. Tumors in weight-bearing bones, such as the legs or spine, may cause the bones to fracture without apparent reason. If the skin is affected, a skin rash, such as cradle cap and nappy rash, may occur. A discharge from the ear or hearing problems can occur if the ear is affected. If LCH affects the lungs or chest, the child may have breathing difficulties.

The lymph glands may be enlarged. Children may be irritable and have a poor appetite. Langerhans cell histiocytosis is treated with corticosteroids, which suppress immune function. Psoralen plus ultraviolet A (PUVA) is another excellent treatment for cutaneous only LCH or for cutaneous disease in multisystemic disease. Radiation therapy or surgery may also be used to treat bone lesions. Bone marrow transplantation represents the ultimate step if a suitable donor can be found. Prevention is better then cure. Avoid smoking is best cure this disorder. Quitting smoking can improve the outcome in people with histiocytosis that affects the lungs.