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Diseases

Genetic and Rare Diseases Information Center (GARD)

Nodular nonsuppurative panniculitis


Other Names for this Disease
  • Weber Christian disease
  • Weber-Christian disease
  • Panniculitis nodular nonsuppurative
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your 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?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

How might nodular nonsuppurative panniculitis be treated?

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.[1] 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.[2][3] 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.[2][3]

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

What is long-term outlook for nodular nonsuppurative panniculitis?

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.[3] The disease tends to recur at intervals of weeks or months.[2] 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.[2][3]
Last updated: 4/6/2016

Where might an individual with nodular nonsuppurative pannicultis go to receive treatment for the condition?

Individuals with nodular nonsuppurative panniculitis (NNP) may consult with a pediatric dermatologist and/or a pediatric rheumatologist in order to confirm the diagnosis, determine the cause of the condition and obtain treatment.[3]

The Society for Pediatric Dermatology, a national organization in the United States specifically dedicated to the field of Pediatric Dermatology, has a "Find a Pediatric Dermatologist" section on its website that can be accessed by clicking here. For the adult population, the American Academy of Dermatology, an organization that represents practicing Dermatologists in the United States, has a "Find a Dermatologist" section on its website that can be accessed by clicking here

The American College of Rheumatology, an organization primarily for physicians, health professionals, and scientists, has a "Find a Rheumatologist" section on its website where individuals can find both pediatric and adult rheumatologists. This this can be accessed by clicking here.
Last updated: 4/6/2016

How can I find an expert who has knowledge and experience regarding a specific condition?

Although there is no list of experts for rare diseases, a fact sheet is available on our Web site with tips for finding healthcare professionals and researchers who have experience with a particular condition. Potential resources include patient advocacy groups, researchers conducting clinical trials, and authors of articles published in medical journals. Click here to view our fact sheet. If you are unable to locate an expert using these suggestions, please let us know.
Last updated: 9/29/2014

References
Other Names for this Disease
  • Weber Christian disease
  • Weber-Christian disease
  • Panniculitis nodular nonsuppurative
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.