Histiocytosis symptoms are changes in the body’s immune system caused by too many white blood cells called histiocytes. These cells normally help the body fight infections and remove debris and other substances that are in the body. But when too many histiocytes develop in a single area of the body, the result is an illness or a tumor.
Langerhans cell histiocytosis (LCH) is one of the most common types of histiocytosis, affecting children and adults of all ages. People with this form of the disease often have lung involvement, and the condition is most likely to start in children or babies between ages 2 and 3.
The cause of LCH is unknown. But researchers think it is caused by a change in a gene (mutation) that normally allows normal white blood cells to develop.
This change leads to too many histiocytes in certain tissues, including the skin, bones, spleen, liver and lungs. The extra histiocytes then cause the tissues to break down. This causes the tumors that are a common symptom of histiocytosis to develop.
Patients with LCH often get better with treatment. However, they may have new lesions or old ones return after treatment is stopped. This is known as reactivation and can occur within 1 year after treatment.
In some cases, new lesions or old ones may come back in the same place as before treatment started, such as the bone, ears or skin. This can be an emergency and may require urgent medical attention.
Signs and symptoms of histiocytosis vary depending on which parts of the body have histiocytes, but they typically include a rash or itchy patches of skin that appear anywhere on the body. This rash can be raised and purple or brown in color, or floppy and flaky. It is accompanied by loss of appetite and fatigue.
Swollen lymph nodes or painful lumps in the neck, armpits, groin and under the arms are other signs of histiocytosis. Abdominal pain, nausea and vomiting are also common.
Hemophagocytic lymphohistiocytosis and malignant histiocytosis are two other types of histiocytosis that can affect multiple organs or the whole body. The symptoms of these conditions vary and may include anemia, weight loss, fever, jaundice, mental deterioration, eye problems, enlargement of the spleen and liver, diarrhea and vomiting.
The first sign of histiocytosis in children is usually a rash that resembles cradle cap and begins on the scalp. The rash can spread to other parts of the body, such as the ear canals or stomach. In severe cases, this rash can cause sepsis and other serious problems.
Other symptoms of histiocytosis include abdominal pain, vomiting and fever. The condition can lead to a short stature and delayed puberty in children. In older children, it can also affect the brain and cause deterioration of neurological function (neurodegeneration).
The signs and symptoms of histiocytosis can range from mild to life-threatening. The best way to diagnose histiocytosis is through a medical evaluation and laboratory tests. Doctors will look at the number of histiocytes in the body and determine which type of histiocytosis is present. The tests may involve blood, x-rays, CT scans or biopsies. The test results are used to make a diagnosis and help physicians decide on the best course of treatment for patients.
