Langerhans Cell Histiocytosis in Children

Langerhans cell histiocytosis (histiocytosis X) is a rare disorder. It occurs when there are very high levels of a type of immune cell called a Langerhans cell. In the past, this disorder was thought to be a type of cancer or a condition similar to cancer. Researchers are now finding that it is more likely tied to an autoimmune response. It happens when the body's immune system attacks itself by mistake.

What is Langerhans cell histiocytosis in children?

Langerhans cell histiocytosis causes damage to tissues all over the body. The extra Langerhans cells are a type of white blood cell. They flow all over the body. They build up in areas such as the skin, bones, bone marrow, lymph nodes, mouth, spleen, liver, lungs, pituitary gland, and central nervous system. This creates tumors.


Anatomy of a bone, showing blood cells
Everyone has Langerhans cells, which are a form of white cell usually found in the lymph nodes, spleen, skin, liver, lungs, and bone marrow. (Click to Enlarge)


What are the symptoms of Langerhans cell histiocytosis in a child?

The symptoms of Langerhans cell histiocytosis depend on where in the body the Langerhans cells build up. These are possible symptoms:

  • Pain in the belly or in the bones   

  • Poor growth (failure to thrive)

  • Irritability

  • Yellowing of the skin and eyes (jaundice)

  • Urinating often and having constant thirst. This is caused by diabetes insipidus.

  • Dark red or brown sores (lesions) on the skin, especially on the face, scalp, back, and groin

  • Bulging eyes

  • Constant drainage from the ear

  • Headaches

  • Flaking skin on the scalp similar to cradle cap

  • Thyroid problems

  • Problems with balance, memory, or behavior

  • Swollen gums, mouth sores, and tooth