Weber-Christian Disease

• 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.

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.

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.

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.

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.

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.

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).

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.