Idiopathic nodular panniculitis is a rare spectrum of skin disorders characterized by single or multiple, tender or painful bumps below the surface of the skin (subcutaneous nodules) that usually lead to inflammation of the subcutaneous layer of fat (panniculitis). These nodules tend to be 1-2 centimeters large and most often affect the legs and feet (lower extremities). In most cases, idiopathic nodular panniculitis is associated with fever, a general feeling of ill health (malaise), muscle pain (myalgia), and/or abdominal pain. These symptoms may subside after a few days or weeks and may recur weeks, months, or years later. The exact cause of idiopathic nodular panniculitis is not known (idiopathic).
Idiopathic nodular panniculitis usually begins gradually. Abnormal bumps or masses (nodules) appear in the fatty layer under the skin (subcutaneous fat) of the legs, thighs, and buttocks. In some cases, the arms, abdomen, and/or face may be involved. These nodules are usually 1-2 centimeters wide and may be either painful and tender or painless. In some cases, the affected area may become red (erythema) and waste away (atrophic), eventually healing and leaving a slight depression.
Another common finding associated with idiopathic nodular panniculitis is recurrent episodes of fever. Additional symptoms include a general feeling of ill health (malaise), fatigue, muscle pain (myalgia), joint pain (arthralgia), and/or abdominal pain. In some cases, weight loss may occur and nausea may be present. In rare cases, inflammation of skin the near the eye (orbital inflammation) may result in abnormal protrusion of the eye (proptosis).
In some cases, inflammation of the subcutaneous layer of fat (panniculitis) may affect additional organ systems of the body (systemic), potentially resulting in blood abnormalities such as low levels of circulating red blood cells (anemia), liver involvement such as an abnormally large liver (hepatomegaly), and lung involvement such as accumulation of fluid in the membrane surrounding the lungs (pleural effusion).
The exact cause of idiopathic nodular panniculitis is unknown. There are numerous different causes that may be associated with the development of panniculitis including gout, diabetes mellitus, systemic lupus erythematosus, subacute bacterial endocarditis, tuberculosis, iodide or bromide therapy, withdrawal from large doses of corticosteroids, or pancreatitis. Sometimes the cause may be identified as an allergy or possibly a predisposition of fatty tissue to a granulomatous reaction.
Idiopathic nodular panniculitis is a rare disorder that may affect males and females of any age group. According to the medical literature, most cases have been young adult females.
The term Weber-Christian disease has been used to describe a group of syndromes or diseases characterized by nodular panniculitis and additional symptoms involving various organ systems of the body (systemic). However, the association of nodular panniculitis and systemic findings may occur due to a variety of causes or secondary to several different diseases, such as systemic lupus erythematous, alpha-1-antitrypsin disease, and connective tissue disease. The term Weber-Christian disease has led to confusion in the medical literature and many researchers suggest that its use be abandoned.
A diagnosis of idiopathic nodular panniculitis is made based upon a detailed patient history, a thorough clinical evaluation, and identification of classic symptoms. In many cases, surgical removal (biopsy) and microscopic examination of small samples of deep skin tissue may reveal inflammation of subcutaneous layers of fat tissue.
Treatment of idiopathic nodular panniculitis is symptomatic and supportive. In some cases, skin lesions may heal spontaneously (remission). However, they often return (recur). Affected individuals should receive a thorough clinical examination to determine whether the idiopathic nodular panniculitis is actually occurring secondary to another condition, as treatment of that primary condition may alleviate the symptoms of idiopathic nodular panniculitis.
In severe cases, corticosteroid treatment (e.g., prednisone) may be effective under controlled conditions (e.g., limited duration).
Treatment of idiopathic nodular panniculitis with oral cyclophosphamide (a cytostatic drug) has shown some promise in preliminary clinical trials. More research is necessary to determine the long-term safety and effectiveness of this treatment for idiopathic nodular panniculitis.
Treatment of idiopathic nodular panniculitis with oral cyclosporin A, an immunosuppressive agent, has shown some promise according to the medical literature. In some cases, treatment with cyclosporin A has led to improvement in symptoms including regression of subcutaneous nodes and overall improvement of health. More research is necessary to determine the long-term safety and effectiveness of this treatment for idiopathic nodular panniculitis.
Researchers are studying the use of an immunosuppressive drug known as mycophenolate mofetil as a potential treatment for individuals with idiopathic nodular panniculitis. Initial reports have suggested that individuals taking this medication have experienced rapid improvement of symptoms. However, more research is necessary to determine the long-term safety and effectiveness of this treatment for individuals with idiopathic nodular panniculitis.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
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