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About Langerhans Cell Histiocytosis

Langerhans cell histiocytosis (LCH) is a group of rare disorders all characterized by the overproliferation of Langerhans cells. Treatment for this condition varies and usually includes a combination of chemotherapy, radiation therapy, corticosteroids and surgery. Some patients may not need any intervention beyond close monitoring.

Normally, Langerhans cells function as part of the immune system and help fight infection, but when Langerhans cells proliferate uncontrollably, they can accumulate in body tissues, causing damage or the formation of a lesion.

LCH can arise anywhere in the body but is found most often in the bones, including the skull and—very rarely—the spine. Other common locations include the skin, spleen, lungs, liver and pituitary gland. LCH may appear either as a single lesion or multiple lesions. When LCH involves the central nervous system, disease is usually also found elsewhere in the body. The focus of this page is LCH of the brain and skull.

Rather than referring to a single disorder, LCH describes a spectrum of diseases whose characteristics and relationships continue to be studied. Currently, the LCH designation includes the following subtypes:

  • Eosinophilic granuloma—This is the most common subtype and is usually found as single or multiple lesions in the skull but can arise in other bones, such as the vertebrae, ribs, mandibles and bones of the arms and legs.
  • Hand-Schüller-Christian disease—This subtype tends to have multiple lesions and is accompanied by diabetes insipidus, exophthalmos and skull lesions.
  • Letterer-Siwe disease—This is the least common but most severe subtype, affecting infants and children younger than age 2. This disease is systemic, involving the whole body, and can be fatal. Symptoms are often most evident on the skin, such as purpuric rash, hemorrhage under the skin and pustules on the skin.
  • Hashimoto–Pritzker disease—This is a congenital condition that typically occurs within the first few months of life and then resolves on its own a few months later. Reddish-brown papules on the head, neck, hands and feet often accompany this disease. The cause of this condition is unclear.

patient journey

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