Types of Histiocytosis

Types of Histiocytosis


The symptoms of different types of histiocytosis are similar. The subtype of disease is often misdiagnosed. A correct diagnosis is important for making sure that the best treatment is offered. There are dozens of subtypes of histiocytosis. These are the most common.

Langerhans Cell Histiocytosis (LCH)

Langerhans cell histiocytosis (LCH) is more common in children, but it can also occur in adults. Most frequently, LCH is a mild illness affecting the skin or only a few bones. However, some people with LCH have tumors in many organs.

LCH of the skin can cause a variety of rashlike conditions. These disorders can be extremely painful. LCH of the skin is often misdiagnosed as another skin disorder until a biopsy (a sample of the tissue) is performed. LCH located only in the skin can occur in children younger than 1 year old. This form of the disease often goes away on its own.

LCH of the bones can cause pain and swelling in the skull, jaw, spine, hips, or other areas.

In rare cases, LCH can affect the brain, causing problems with balance, speech, and coordination. These effects are similar to those seen in Erdheim-Chester disease.

LCH that affects limited parts of the body may be treated with localized treatments. These include the removal of tumors and skin treatments. More-extensive LCH may require chemotherapy, radiation, or drugs that target particular gene mutations.

Erdheim-Chester Disease (ECD)

Erdheim-Chester disease (ECD) is a type of histiocytosis that mainly occurs in adults. Adolescents and children are rarely affected. Like all histiocytic diseases, ECD can be challenging to diagnose because it can affect any part of the body and causes a wide variety of symptoms. A biopsy (a sample of the tissue) can be difficult to interpret.

Nearly all people with ECD have tumors in their leg bones. About half of them also have pain in the legs or other bones. General symptoms, such as fatigue and weakness, are extremely common in ECD. Problems with balance, thinking, memory, and mood are frequent too. ECD is more likely than the other histiocytic diseases to affect the kidneys, heart, skin around the eyelids, and area behind the eyes.

Some people with ECD have mild illness. Others have severe symptoms and difficulty performing normal daily activities. ECD is most serious when it affects the brain. This can cause problems with walking, speech, coordination, and other issues. Treatment is almost always needed for ECD.

Rosai-Dorfman Disease (RDD)

Rosai-Dorfman disease (RDD) is usually found in children but can also affect adults. RDD can happen in people who have autoimmune diseases or other blood cancers.

RDD usually causes swollen lymph nodes, but it can affect any part of the body. After the lymph nodes, the most frequently affected areas of the body are the bones and skin. The brain and other areas can also be affected.

Some forms of RDD do not require treatment and can improve on their own. For those that do not, treatments include surgery, steroid medications, and in some cases, chemotherapy or targeted drugs.

Juvenile Xanthogranuloma (JXG)

Juvenile xanthogranuloma (JXG) is a histiocytosis that occurs in children and adolescents. It primarily affects the skin, but in rare circumstances it can affect other areas, including the bones, the area behind the eyes, and the brain. JXG that is localized to the skin can occur in infants. When this happens, it usually goes away on its own.

Adult xanthogranuloma is a similar histiocytosis that affects adults.

Malignant Histiocytic Disorders

Malignant histiocytoses are very rare and often aggressive histiocytic diseases. These include histiocytic sarcoma, Langerhans cell sarcoma, and others. They can affect the skin, lymph nodes, brain, and other organs.

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