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- Weber-Christian disease facts
- 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 facts
- Weber-Christian disease is an uncommon inflammatory condition of the fatty tissues of the body.
- It's a disease of unknown cause.
- It most commonly affects the thighs and legs of women.
- It's difficult to treat and may heal with permanent scars.
What is Weber-Christian disease? What are the risk factors for this disease?
Weber-Christian disease is an uncommon inflammatory disease of the fatty tissues of the body. Weber-Christian disease is also referred to as idiopathic lobular panniculitis (ILP) and relapsing febrile nodular panniculitis syndrome.
The term Weber-Christian often refers to a group of diseases that involve the deep fat. These disorders first appear on the skin as reddish, tender nodules. Mostly, Weber-Christian describes cases of recurring inflammation of special sections of fat called lobules. The exact cause of Weber-Christian panniculitis is unknown. The disorder is frequently associated with systemic symptoms such as fever and body aches.
Weber-Christian disease is most commonly seen in females 30-60 years of age. It can occur in both sexes, in all ages, and rarely in infants. There are no other known risk factors.
Usually, both sides of the body are affected, and the thighs and lower legs are the most frequent areas. The inflamed areas can lose their blood supply, the skin can actually die in the area, ooze yellowish drainage, and become infected. Scarring is common.
The appearance of the skin symptoms are usually accompanied by fever and malaise (feeling poorly). The skin lumps may vary in size but are usually small, the size of two to three fingertips or about 1-2 cm in size. The skin lumps may gradually flatten, soften, and start to decrease over several weeks. Frequently, the areas heal with a brownish or tan discoloration and leave a sunken scar from the underlying fat necrosis. Less commonly, the skin discoloration may take weeks to months to fade away completely and leave no scar.
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) 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 disease. 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.
What is the prognosis of Weber-Christian disease?
The prognosis or long-term outlook is very different for each patient with Weber-Christian disease and depends on the severity of organ involvement. Some patients have mild, intermittent, annoying symptoms, while in others Weber-Christian disease can be fatal. After several years of symptoms, Weber-Christian disease may go away permanently (remission).
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What is the treatment for Weber-Christian disease?
There is no cure or uniformly effective treatment that works for everyone with Weber-Christian disease. Possible treatments include oral medications that alter the immune-system reaction and decrease overall inflammation. Some patients have had improvement with medications including chloroquine, thalidomide, cyclophosphamide, tetracycline, cyclosporine, azathioprine, prednisone, and a host of nonsteroidal medications like ibuprofen and indomethacin.
Accompanying treatments for the symptoms may include additional oral pain medications as well as topical salves to treat and prevent local skin infections.
Overall, when internal organs are inflamed, medicines directed toward the underlying inflammation are considered. In summary, treatment for Weber-Christian disease is nonspecific, and antiinflammatory therapy may not be fully effective for everyone with the disease.
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Lee, Lela, M.D. "Panniculitis: Recognition and diagnosis." UptoDate. Updated Apr. 27, 2016.
Weber-Christian Disease - Symptoms
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Alpha 1 Antitrypsin Deficiency
Alpha-1 antitrypsin deficiency (AATD) is an inherited disorder caused by mutations in the SERPINA1 gene. People with the condition are at risk for developing serious lung and liver disease. Symptoms and signs of lung disease caused by this condition include:
The earliest symptoms and signs of lung disease usually develop between 20 and 50 years of age, and are
- The reduced ability to exercise
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Liver disease: Alpha-1 antitrypsin deficiency also cause liver disease in some people with the condition, that include liver cancer, cirrhosis of the liver, an abnormally large liver (hepatomegaly), liver failure, and hepatitis. Liver damage from alpha-1 antitrypsin deficiency causes symptom of a swollen abdomen, swollen legs or feet, and jaundice.
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Complete Blood Count
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cyclophosphamideCyclophosphamide (Cytoxan) is a drug prescribed for the treatment of certain cancers including breast cancer, leukemia, and ovarian cancer. Side effects, drug interactions, patient safety information, and pregnancy and breastfeeding information should be reviewed prior to taking any medication.
Ibuprofen is a nonsteroidal anti-inflammatory drug (NSAID) used to reduce mild to moderate pain, inflammation, and fever. Ibuprofen works by blocking an enzyme that makes prostaglandin (a hormone-like substance that participates in a variety of body functions), which results in lower levels of prostaglandins in the body. Lower levels of prostaglandins reduce pain, inflammation, and fever.
Ibuprofen is prescribed to treat diseases and conditions that cause mild to moderate pain, fever, and inflammation. For example, Pain from strains and sprains; pain from cuts, scrapes, and puncture wounds; muscle aches and pains; tooth pain; common cold; mild headache; some arthritis conditions; joint pain; and to reduce fever.
Common side effects of ibuprofen include, diarrhea, constipation, nausea, heartburn, belly pain, drowsiness, headaches, tinnitus (ringing in the ears), and mild rash.
More serious side effects and adverse effects include, increased bleeding after injury, stomach ulcers, impaired kidney function, severe allergic reaction (anaphylaxis), blood clots, heart attack, heart failure, and high blood pressure.
The maximum dose prescribed under a doctor's care is 3.2 g daily. Otherwise, the over-the-counter (OTC) maximum daily dose is 1.2 g daily. Dosage depends upon the age, weight, and any current medical conditions of the patient. Several drugs interact with ibuprofen so check with your doctor, pharmacist, or other health care professional with questions in regard to this drug. Doctors don't know if it is safe to take ibuprofen if your are pregnant, therefore it is not recommended if you are pregnant. According to the American Academy of Pediatrics, ibuprofen is safe to take while breastfeeding.
REFERENCE: FDA Prescribing Information.
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