Symptoms
The aggressiveness of the disease seems to be related to the age of the patient.
In children under the age of 2, there is often widespread involvement of the
skin, resulting in a seborrhoeic rash. The scalp and the skin behind the ears
are most characteristically involved. In addition, there is frequent swelling
of the lymph glands. The liver and the spleen are usually enlarged, giving rise
to a distended abdomen. There may be anaemia due to bone marrow involvement,
and shortness of breath due to lung involvement.
In older children between the ages of 2 and 8, the disease tends to be less
aggressive and usually involves the bone and soft tissue. Any bone in the body
may be involved, but the skull bone is frequently eroded and the external ear
is often inflammed. Sometimes, the disease affects the secretion of hormone
of the pituitary gland and then the child passes a lot of urine and is thirsty
all the time. This is called diabetes insipidus.
In children above the age of 8, the disease is usually localized and appears
as single or multiple bone lesions.