Weber-Christian Disease

What is Weber-Christian Disease?

Weber-Christian disease is also known as idiopathic relapsing febrile non-suppurative panniculitis and is a cutaneous condition characterized by subcutaneous nodules, inflammatory cells in the fat lobules, and systemic symptoms.

It is an uncommon disease seen in women 30-60 years of age.  Lesions are usually seen in the lower legs and are bilaterally symmetrical.  The disease is named after Frederick Parkes Weber and Henry Asbury Christian and is believed to be a T-cell mediated autoinflammatory condition.

Weber-Christian disease is rare in adults and even rarer in children and may involve the heart, lungs, spleen, intestines, adrenal glands, kidney, and orbits.  The condition occurs more often among women who make up 75% of reported cases.  A skin biopsy is necessary to confirm the diagnosis of panniculitis.

What are the Symptoms of Weber-Christian Disease?

The disease causes crops of lesions that appear and go away over a period of weeks and months. The lesions are often symmetric and appear on the legs and thighs.

Systemic symptoms include:

Nausea, fever, malaise, abdominal pain, vomiting, bone pain, weight loss, arthralgia, and myalgia.

Weber-Christian Disease Causes

Doctors do not know what causes Weber-Christian Disease (or idiopathic lobular panniculitis) at this time. They suspect, though, that it is an immune-mediated disease – meaning that the immune system senses something is wrong and reacts to it.

Research studies have focused on the disease potentially being caused by a reaction to an infection, but they have not found anything conclusive yet after exploring that particular avenue.

Known risk factors for this condition include:

  • Gender – three-quarters of the cases diagnosed are women and young girls.
  • Age – while it occurs in children and young adults, it appears most often in women between the ages of 40-69.

Race is not thought to be a risk factor for Weber-Christian Disease. It does not seem to be an inherited condition, and is not any more or less prevalent in developing nations versus developed nations, but that may say more about the lack of information about the disease than it does about where it is more likely to happen.

While it is known that the majority of cases occur in women and young girls, no one is sure why this would be the case. Studies are ongoing.

How is a Weber-Christian Disease Treated?

Non-steroidal anti-inflammatory agents may reduce arthralgias, fever, and other signs of malaise but there is no single effective therapy for Weber-Christian disease.

The use of corticosteroids and immunosuppressive agents have shown promise. One patient improved rapidly after being given the powerful immunosuppressive agent Cyclosporin A and receiving corticosteroid treatment.

Weber-Christian Disease Prevention

With no known causes, it is difficult to prevent Weber-Christian Disease. The condition is considered rare, with Mayo Clinic only discovering 30 cases of it between 1960 and 1998 (a 28 year time span). Part of this is due to information about the disease that is ambiguous, at best. More people may have it than are known about because they have been diagnosed with other conditions that have similar symptoms and occur more often.

Weber-Christian disease is considered a chronic condition, so prevention efforts focus more on managing the symptoms as they appear. The management plan includes anti-inflammatory medications to treat inflammation in the internal organs.

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