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Hand-Schuller-Christian disease causes lipid buildup, forming masses in various parts of the body. Symptoms vary widely and can include anemia, bone marrow damage, and breathing problems. Treatment depends on individual symptoms and may include medication, radiation, or surgery. The cause is not clearly understood, and symptoms usually appear by age six. Blood disorders, bone defects, and diabetes insipidus are common. Diagnosis can be difficult due to the wide range of symptoms. Treatment may involve individual symptom management, chemotherapy, radiation, or surgery.
Hand-Schuller-Christian disease is a rare disease that causes lipids to build up within the body. These lipids clump with immune cells known as histiocytes to form masses known as granulomas in various parts of the body, including the skin, skull and many of the internal organs. Symptoms of Hand-Schuller-Christian disease vary widely and depend on the size and location of the tumors. Some of the more common symptoms include anemia, bone marrow damage, and breathing problems. Treatment depends on the individual symptoms present and may include the use of medications, radiation therapy, or surgery.
The exact cause of Hand-Schuller-Christian disease is not clearly understood, and a variety of blood disorders can be present. Symptoms usually appear by age six, with boys being affected slightly more often than girls. Bone defects and a type of diabetes known as diabetes insipidus are commonly found among those with this disease. An abnormal bulging of the eye is also seen in most of those with this condition.
Symptoms of Hand-Schuller-Christian disease vary widely from patient to patient, often leading to difficulty getting an accurate diagnosis. Blood-related problems are common and can include anemia, blood cell abnormalities, or excessive bleeding. Skin problems can also develop, often leading to the development of an unexplained rash or raised areas of skin that may be discolored, flaky, or greasy. Swollen gums and early tooth loss are also common in those with this disease.
Some of the more serious potential symptoms of Hand-Schuller-Christian disease can include developing diabetes, thyroid dysfunction, or difficulty breathing. Sometimes swollen lymph nodes or damage to the pituitary gland can occur. Frequent infections, visual disturbances, or varying degrees of neurological damage are also sometimes observed. Due to the wide range of possible symptoms and the fact that it is a very rare disease, it can sometimes take several months or more before doctors are able to make an accurate diagnosis.
Treatment for Hand-Schuller-Christian disease usually begins with treating individual symptoms, such as prescribing iron supplements for someone with anemia. It may be necessary to resort to more intensive treatments, such as chemotherapy or the use of radiation, in some of the more severe cases. Surgery can be used in an attempt to remove large tumors or to repair any internal damage caused by the presence of this disease.
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