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Last updated:
August 18, 2021
Years published: 1986, 1989, 1992, 1996, 1997, 2000, 2001, 2005, 2007, 2021
NORD gratefully acknowledges Nicole Kuriki, PharmD Candidate, NORD Editorial Intern from Keck Graduate Institute, and Quintin Broussard, PharmD, BCPS, BCCCP, BCNSP, Assistant Professor of Clinical Sciences at Keck Graduate Institute School of Pharmacy and Health Sciences, for assistance in the preparation of this report.
Summary
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 can be open sores (ulcerated) and usually lead to inflammation of the subcutaneous layer of fat (panniculitis). These nodules tend to be 1-2 centimeters in size and most often affect the legs and feet (lower extremities). Rare cases involve panniculitis of the breast. In most patients, 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 and on multiple occasions. The exact cause of idiopathic nodular panniculitis is not known (idiopathic).
Introduction
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.
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 patients, 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 patients, the affected area may become blue and red (erythema), ulcerated (open sore with yellow discharge) and waste away (atrophic), eventually healing and leaving a slight depression after a few weeks.
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 patients, weight loss may occur and nausea may be present. Rarely, inflammation of skin near the eye (orbital inflammation) may result in abnormal protrusion of the eye (proptosis).
In some patients, 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), inflammation of blood vessels (vasculitis), sore muscles and joints (polyarthralgia and polymyalgia), piercing of the bowels (perforation) and lung involvement such as accumulation of fluid in the membrane surrounding the lungs (pleural effusion). These systemic events can be life threatening.
The exact cause of idiopathic nodular panniculitis is unknown. There are numerous different causes that may be associated with the development of panniculitis including Sjogren’s syndrome, inflammatory bowel disease, 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 inflammation following cell injury due to an infection or toxin (granulomatous reaction). One hypothesis of the cause of disease is that fat is the trigger of an autoimmune reaction, causing white blood cells to invade the area and cause pathological damage to the subcutaneous fat resulting in the nodules.
Idiopathic nodular panniculitis is a rare disorder that may affect males and females of any age group but rarely affects young children. Most cases are young adult females.
A diagnosis of idiopathic nodular panniculitis is made based upon a detailed patient history, thorough clinical evaluation and identification of classic symptoms. In many patients, surgical removal (biopsy) and microscopic examination of small samples of deep skin tissue may reveal inflammation of subcutaneous layers of fat tissue. Diagnosis of nodular panniculitis can be made after all other forms of lobular panniculitis have been excluded.
Laboratory tests for differentiation and exclusion of other diseases include C-reactive protein, serum protein electrophoresis, erythrocyte sedimentation rate, alpha-antitrypsin, pancreatic enzymes, angiotensin-converting enzyme and rheumatoid factor.
Treatment
Treatment of idiopathic nodular panniculitis is symptomatic and supportive. In some patients, 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.
Non-severe cases that do not include other body systems are treated with pain relieving analgesics (e.g. NSAIDS). If ulcers are present, dressing should be applied to reduce likelihood of infection. For supportive care in patients with severe, uncontrolled pain, opioids may be used. Initial therapy for severe cases includes surgical excision of the nodule and systemic therapy such as an antibiotic (e.g. dapsone, ceftriaxone), immunosuppressant (e.g., azathioprine) or the antimalarial agent hydroxychloroquine. Additional corticosteroid treatment (e.g. prednisone) may be effective under controlled conditions (e.g. limited duration).
Treatment of idiopathic nodular panniculitis with oral cyclophosphamide (a cell growth and division inhibitor, also known as a cytotoxic 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 patients, 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.
Success of remission in one patient has been found using the phendimetrazine tartrate derivative antibiotic clofazimine, with slow tapering over three months. Clofazimine is a known treatment to similar diseases such as leprosy and erythema nodusum leprosum.
There are currently no ongoing clinical trials for idiopathic nodular panniculitis.
Information on current clinical trials is posted on the Internet at https://clinicaltrials.gov/ All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
https://www.centerwatch.com/
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
JOURNAL ARTICLES
Rotondo C, Corrado A, Mansueto N, et al. Pfeifer-Weber-Christian disease: a case report and review of literature on visceral involvements and treatment choices. Clin Med Insights Case Rep. 2020;13:1-8.
Zheng W, Song W, Wu Q, Yin Q, Pan C, Pan H. Analysis of the clinical characteristics of thirteen patients with Weber-Christian panniculitis. Clin Rheumatol. 2019; 38(12):3635-3641.
Singh, T., Prabhakar, N., Singla, V., Singh, G. and Nada, R., Imaging of idiopathic nonsuppurative nodular panniculitis of breast. Breast J. 2019;26(3):536-538.
Miranda-Bautista J, Fernández-Simón A, Pérez-Sánchez I, Menchén L. Weber-Christian disease with ileocolonic involvement successfully treated with infliximab. World J Gastroenterol. 2015;21(17):5417-5420.
Mavrikakis I, Georgiadis T, Fragiadaki K, Sfikakis PP. Orbital lobular panniculitis in Weber-Christian disease: sustained response to anti-TNF treatment and review of the literature. Surv Ophthalmol. 2010;55(6):584-9.
Al-Niaimi F, Clark C, Thorrat A, Burden AD. Idiopathic lobular panniculitis: remission induced and maintained with infliximab. Br J Dermatol. 2009;161(3):691-2.
Bukhari I. Idiopathic nodular panniculitis in Niemann-Pick disease. Journal of the European Academy of Dermatology and Venereology: JEADV. 2005;19(5):600-602.
Abuzahra F, Kovacs S, Beermann T, Merk HF, Frank J. Treatment of relapsing idiopathic nodular panniculitis with clofazimine. Br J Dermatol. 2005;152(3):582-3.
Kovacs M, et al. Successful treatment of Weber-Christian panniculitis with cyclosporin-A. Orv Hetil. 2004;145:827-31.
Baskan EB, et al. Effective treatment of relapsing idiopathic nodular panniculitis (Pfeifer-Weber-Christian disease) with mycophenolate mofetil. J Dermatolog Treat. 2003;14:57-60.
Hyun SH, et al. Weber-Christian disease presenting with proptosis: a case report. J Korean Med Sci. 2000;15:247-50.
Iwasaki T, et al. Successful treatment of a patient with febrile, lobular panniculitis (Weber-Christian disease) with oral cyclosporin A: implications for pathogenesis and therapy. Intern Med. 1999;38:612-14.
Miyasaki N. Steroid-resistant Weber-Christian disease. Intern Med. 1999;38:522.
White JW Jr, et al. Weber-Christian panniculitis: a review of 30 cases with this diagnosis. J Am Acad Dermatol. 1998;39:56-62.
Asauliuk IK, et al. Pfeifer-Weber-Christian disease with 20-year course. Lik Sprava. 1998;7:154-59.
Enk AH, et al. Treatment of relapsing idiopathic nodular panniculitis (Pfeiffer-Weber-Christian disease) with mycophenolate mofetil. J Am Acad Dermatol. 1998;39:508-09.
Cook JN, et al. Proptosis as the manifesting sign of Weber-Christian disease. Am J Ophthalmol. 1997;124:125-26.
Viravan S, et al. Successful treatment of cytophagic histiocytic panniculitis by cyclosporin A: a case report. Asian Pac J Allergy Immunol. 1997;15:161-66.
Khan GA, et al. Recognizing Weber-Christian disease. Tenn Med. 1996;89:447-49.
White WL, et al. Panniculitis: recent developments and observations. Semin Cutan Med Surg. 1996;15:278-99.
Biasi D, et al. Weber-Christian disease with calcinosis: a case report. Clin Rheumatol. 1996;15:624-25.
Kumagai-Kurata N, et al. Idiopathic lobular panniculitis with specific pleural involvement. Eur Respir J. 1995;8:1613-15.
Akama H, et al. Glucocorticoid-unresponsive fever in a patient with Weber-Christian disease. Br J Clin Pract. 1994;48:161-62.
Ohara S, et al. Myalgia as the major symptom in systemic panniculitis (Weber-Christian disease). Eur Neurol. 1992;32:321-26.
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