Category: 

What Is Hand-Schuller-Christian Disease?

Article Details
  • Written By: Meshell Powell
  • Edited By: Melissa Wiley
  • Last Modified Date: 17 September 2014
  • Copyright Protected:
    2003-2014
    Conjecture Corporation
  • Print this Article
Free Widgets for your Site/Blog
There is enough iron in the human body to make a nail.  more...

September 22 ,  1862 :  US President Abraham Lincoln announced his preliminary Emancipation Proclamation.  more...

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.

Ad

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.

Ad

More from Wisegeek

You might also Like

Discuss this Article

gsx600
Post 5

I was diagnosed with this at six months old in 1965. I had radiology, bone marrow transplants, blood transfusions and probably more. My life expectancy was only two years, then it went to four, then to 10 and at 16, they gave up and said who knows? I'm now 48 and reasonably fit and healthy, but still suffer from the diabetes insipidus and take a medicine (DDAVP) daily for this.

anon333946
Post 4

My husband had a twin brother diagnosed with this. The symptoms were evident at around 2 years of age. He died before he was 4 years old.

ddljohn
Post 3

@SarahGen-- I think sometimes this condition is misdiagnosed, because the granulomas look just like regular tumors.

SarahGen
Post 2

@anamur-- Hand-Schuller-Christian syndrome is sometimes referred to as Langerhans cell Histiocytosis, so you might want to search for this name instead for your research.

Although I don't know how many people in the US have it, I do know that it's a rare disease. This probably explains the limited amount of information on it.

I believe that the tumors associated with the disease are benign. Lipids are fat, so lipomas in general are benign masses. Granulomas are not exactly tumors, they're more like inflamed masses of cells.

But it's not the malignancy or the benign nature of these tumors that cause problems. It's the fact that they keep forming in the body, in bone marrow and the lymphatic system and lead to many other conditions that harm the body. That's why chemotherapy and surgery is sometimes necessary to remove them.

serenesurface
Post 1

I'm doing a research paper on this disease but it's difficult to find information on it, so thank you for this article.

I'm interested in a few more details though. If anyone has the answer to these, that would be very helpful.

Are the tumors caused by Hand-Schuller-Christian disease malignant or benign?

How does this disease affect an individual's life expectancy? And how often is the disease seen in the American population?

Post your comments

Post Anonymously

Login

username
password
forgot password?

Register

username
password
confirm
email