Hand-Schuller-Christian disease is a rare disorder that causes lipids to develop inside the body. These lipids group together 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 most 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 surgical intervention.
The exact cause for Hand-Schuller-Christian disease is not clearly understood, and a variety of blood disorders may be present. Symptoms usually appear by the age of six years, with males being affected slightly more often than females. Bone defects and a type of diabetes known as diabetes insipidus are commonly found among those with this disease. An abnormal protrusion of the eyes is seen in the majority of those with this condition as well.
The symptoms of Hand-Schuller-Christian disease vary widely from patient to patient, often leading to difficulty in obtaining an accurate diagnosis. Blood-related problems are commonplace and may include anemia, blood cell abnormalities, or excessive bleeding. Skin problems may develop as well, often leading to the development of an unexplained rash or raised areas of skin that may be discolored, scaly, or greasy in appearance. Swollen gums and early tooth loss are frequently seen in those with this disease as well.
Some of the more serious potential symptoms of Hand-Schuller-Christian disease may include the development of diabetes, thyroid dysfunction, or difficulty breathing. Enlarged lymph nodes or damage to the pituitary gland may sometimes occur. Frequent infections, visual disturbances, or varying degrees of neurological damage are sometimes seen as well. Due to the wide range of possible symptoms and the fact that this is a very uncommon disease, it may sometimes take several months or longer for the doctors to be able to make an accurate diagnosis.
Treatment for Hand-Schuller-Christian disease usually begins with treating individual symptoms, such as prescribing iron supplements for those with anemia. More intensive treatment, such as chemotherapy or the use of radiation, may need to be used in some of the more severe cases. Surgical intervention may be used in an effort to remove large tumors or to repair any internal damage caused by the presence of this disease.
