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  3. Nodular nonsuppurative panniculitis
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Nodular nonsuppurative panniculitis


Title




Other Names:
Panniculitis nodular nonsuppurative; Weber-Christian panniculitis; Idiopathic nodular panniculitis; Panniculitis nodular nonsuppurative; Weber-Christian panniculitis; Idiopathic nodular panniculitis; Relapsing febrile nodular nonsuppurative panniculitis; Pfeiffer-Weber-Christian syndrome See More
Categories:
Skin Diseases

Summary Summary


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Nodular nonsuppurative panniculitis describes a rare group of skin disorders characterized by tender, 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 in length and most often affect the legs and feet. In most people, this condition is associated with fever, a general feeling of ill health (malaise), muscle pain, 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 nodular nonsuppurative panniculitis is unknown.[1]
Last updated: 11/8/2014

Symptoms Symptoms


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This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 15 |
Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Abdominal pain
Pain in stomach
Stomach pain
[ more ]
0002027
Aplasia/Hypoplasia of the skin
Absent/small skin
Absent/underdeveloped skin
[ more ]
0008065
Arthralgia
Joint pain
0002829
Edema
Fluid retention
Water retention
[ more ]
0000969
Erythema 0010783
Fever 0001945
Myalgia
Muscle ache
Muscle pain
[ more ]
0003326
Nausea and vomiting 0002017
Panniculitis
Inflammation of fat tissue
0012490
Subcutaneous nodule
Firm lump under the skin
Growth of abnormal tissue under the skin
[ more ]
0001482
Weight loss 0001824
5%-29% of people have these symptoms
Autoimmunity
Autoimmune disease
Autoimmune disorder
[ more ]
0002960
Hepatomegaly
Enlarged liver
0002240
Inflammatory abnormality of the eye 0100533
Splenomegaly
Increased spleen size
0001744
Showing of 15 |
Do you have more information about symptoms of this disease? We want to hear from you.
Last updated: 2/1/2021
Do you have updated information on this disease? We want to hear from you.

Treatment Treatment


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Treatment for nodular nonsuppurative panniculitis (NNP) generally aims at controlling and relieving the symptoms that an individual has. Before treatment is initiated, a work-up should be completed to determine whether the condition is secondary to another underlying disorder. If there is an underlying disorder, treatment of this disorder may relieve the symptoms of NNP. In some cases, skin lesions heal spontaneously (remission) but the lesions often later return.[2] There is no treatment method found to be effective for all individuals with NNP. Medications used to treat the condition may include systemic steroids (such as prednisone) to suppress sudden attacks; nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce fever and other signs of malaise; and/or immunosuppressive drugs.[3][4] Relief of symptoms in some affected individuals has also been reported with fibrinolytic agents (medications that help prevent blood clots), hydroxychloroquine, azathioprine, thalidomide, cyclophosphamide, tetracycline, cyclosporin, mycophenolate, and clofazimine.[3][4]

More detailed information about the management of nodular nonsuppurative panniculitis is available on the Treatment and Medication sections of the Medscape Reference Web site.
Last updated: 4/6/2016

Prognosis Prognosis


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It is not possible to predict the prognosis for specific individuals (including when and if remission will occur), as the prognosis widely varies in individuals with the condition. Those with primarily cutaneous (skin) involvement may experience periods of exacerbations and remission of symptoms with minimal involvement of other organs for several years before the disorder resolves.[4] The disease tends to recur at intervals of weeks or months.[3] In individuals with severe involvement of the heart, lungs, intestines, spleen, kidney, or adrenal glands, morbidity and mortality are significant and these individuals may not survive.[3][4]
Last updated: 4/6/2016

Related Diseases Related Diseases


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Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Differential diagnosis includes erythema nodosum and erythema induratum of Bazin, which represent the commonest forms of predominantly septal panniculitis and predominantly lobular panniculitis with vasculitis, respectively, as well as a variety of distinctive disease entities associated with panniculitis. The latter comprise bacterial, viral and fungal infections, connective tissue disorders, disturbances of lipid metabolism, pancreatic and renal diseases, gout, lymphoproliferative neoplasia, trauma, alpha1-antitrypsin deficiency (see this term).
Visit the Orphanet disease page for more information.

Learn More Learn More


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These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Nodular nonsuppurative panniculitis. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers


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Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • Where and who do I turn to get the help that my daughter needs? What medications are used for this? How long before it will go into remission? How much can be expected of a person that has this and it is not in remission? See answer


Have a question? Contact a GARD Information Specialist.

References References


  1. Panniculitis, Idiopathic Nodular. National Organization for Rare Disorders (NORD). 2007; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/297/viewAbstract. Accessed 8/30/2011.
  2. Panniculitis, Idiopathic Nodular. NORD. July 23, 2007; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/297/viewAbstract. Accessed 4/6/2016.
  3. Nodular non-suppurative panniculitis. Orphanet. March 2007; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=33577. Accessed 4/6/2016.
  4. Eyal Muscal. Weber-Christian Disease. Medscape Reference. Jan 22, 2015; http://emedicine.medscape.com/article/1008411-overview.
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