Nodular nonsuppurative panniculitis

Title

Other Names:

Weber Christian disease; Weber-Christian disease; Panniculitis nodular nonsuppurative

Categories:

Skin Diseases

Summary Summary

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

The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.

Signs and Symptoms	Approximate number of patients (when available)
Aplasia/Hypoplasia of the skin	Very frequent (present in 80%-99% of cases)
Arthralgia	Very frequent (present in 80%-99% of cases)
Edema	Very frequent (present in 80%-99% of cases)
Erythema	Very frequent (present in 80%-99% of cases)
Fever	Very frequent (present in 80%-99% of cases)
Myalgia	Very frequent (present in 80%-99% of cases)
Nausea and vomiting	Very frequent (present in 80%-99% of cases)
Panniculitis	Very frequent (present in 80%-99% of cases)
Subcutaneous nodule	Very frequent (present in 80%-99% of cases)
Weight loss	Very frequent (present in 80%-99% of cases)
Autoimmunity	Occasional (present in 5%-29% of cases)
Hepatomegaly	Occasional (present in 5%-29% of cases)
Inflammatory abnormality of the eye	Occasional (present in 5%-29% of cases)
Splenomegaly	Occasional (present in 5%-29% of cases)

Last updated: 10/1/2017

Treatment Treatment

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

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

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Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Providing General Support

American Skin Association, Inc.
346 Park Avenue South, 4th floor
New York, NY 10010
Toll-free: 800-499-SKIN
Telephone: 212-889-4858
Fax: 212-889-4959
E-mail: info@skinassn.org
Website: http://www.americanskin.org

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Learn More Learn More

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.

News & Events News & Events

News

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