Symptoms of LCH vary widely from a benign or mild form to a very aggressive, even fatal disease. Symptoms can develop at any age, but it is more common in children than adults. The most severe cases tend to occur in children under 2 years of age.
Symptoms depend on the organs of the body affected. Bones are a common site, occurring as a single lesion or many lesions. The lesions of the bones are usually found because of pain and swelling in the affected areas including the skull, femur in the leg, ribs, pelvis, vertebrae and jaw.
The skin and scalp may also be involved. Skin lesions may include small sacs or blisters containing pus, small reddish elevations or bumps on the skin, scaly / greasy rashes, and purplish-red spots or bleeding under the skin. Swelling of the gums, early eruption of the teeth, and loss of teeth may occur. In addition, there may be draining from the ears.
When the hypothalamic / pituitary area of the brain is affected, diabetes insipidus may occur. The pituitary is located at the base of the brain. It produces a hormone called vasopressin that helps the body save water. When a mass of histiocytes gets in the way, hormone production is reduced or stopped. When this happens, the affected person is thirsty and has increased urination. Involvement of this area may also cause growth failure and thyroid problems.
LCH may also affect the lungs, causing breathing problems due to the disease or from an infection. Lung involvement, however, is more often seen in adults with a history of smoking. The bone marrow is affected when the histiocytes multiply in the marrow and crowd out normal red cells, white cells and platelets. A decrease in these cells may cause anemia and fatigue due to low red blood cells, increased infections due to low numbers of white blood cells, and the tendency to bruise easily and/or develop petechiae due to low numbers of platelets.