Weber-Christian Disease (cont.)
Nili N. Alai, MD, FAAD
Dr. Alai is an actively practicing medical and surgical dermatologist in south Orange County, California. She has been a professor of dermatology and family medicine at the University of California, Irvine since 2000. She is U.S. board-certified in dermatology, a 10-year-certified fellow of the American Academy of Dermatology, and Fellow of the American Society of Mohs Surgery.
William C. Shiel Jr., MD, FACP, FACR
Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.
In this Article
- What is Weber-Christian disease? What are the risk factors for this disease?
- What is panniculitis?
- What are other symptoms of Weber-Christian disease?
- What causes Weber-Christian disease?
- How is Weber-Christian disease diagnosed?
- What is the prognosis of Weber-Christian disease?
- What is the treatment for Weber-Christian disease?
- Weber-Christian Disease At A Glance
- Find a local Dermatologist in your town
What is panniculitis?
The tissue layer under the skin (epidermis and dermis) is called the subcutaneous fat or panniculus. This subcutaneous tissue is very important in temperature regulation and protective insulation of the body. Inflammation of this essential layer of fatty tissue is called panniculitis. In panniculitis, the overlying skin typically appears as red or purplish lumps.
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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