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February 10, 2012

Weber-Christian Disease (cont.)

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What are other symptoms of Weber-Christian disease?

Weber-Christian disease can also cause symptoms other than in the skin, such as fever, fatigue, nausea, vomiting, weight loss, and joint pain. Occasionally, inflammation occurs in other organs of the body to cause heart, lung, kidney, liver, and/or spleen problems. Liver involvement may first cause abdominal pain. The skin symptoms provide perhaps the most important clue to the diagnosis of Weber-Christian syndrome. Overall, symptoms with this syndrome may come and go, and relapses are common.

What causes Weber-Christian disease?

The cause of Weber-Christian disease or idiopathic lobular panniculitis is not known. Idiopathic means unknown cause. A misdirected immune reaction may play a role. The cause may be related to an abnormal bodily response to the normal inflammation.

How is Weber-Christian disease diagnosed?

The diagnosis is made usually by taking a piece of tissue (biopsy) and examining it under a microscope. There is inflammation of the affected fatty lobules (central part of the fat) of body with increased numbers of white blood cells around the fat and sometimes degeneration or death of fat cells called necrosis.

Laboratory tests using a simple blood draw may be helpful as extra information to suggest inflammation when Weber-Christian disease is suspected. There is no single lab test that completely defines this diagnosis. Abnormal tests such as a highly elevated erythrocyte sedimentation rate, also called "sed rate" or ESR, may be useful. Additionally, mildly elevated white blood cell count (WBC) on a complete blood cell count (CBC) may be found.

Normal lab tests, including serum and urine amylase and lipase, help to distinguish Weber-Christian from other diseases of the fat caused by pancreatitis (inflammation of the pancreas). A normal alpha-1 antitrypsin level helps to differentiate Weber-Christian disease from a separate fatty inflammatory disease (panniculitis) caused by alpha-1 antitrypsin deficiency.



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