Rare Endocrinology News
Disease Profile
Nodular nonsuppurative panniculitis
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Unknown
Age of onset
Adult
ICD-10
M35.6
Inheritance
Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.
Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.
X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Not applicable
Other names (AKA)
Panniculitis nodular nonsuppurative; Weber-Christian panniculitis; Idiopathic nodular panniculitis;
Categories
Skin Diseases
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]
Symptoms
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.
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 |
Treatment
More detailed information about the management of nodular nonsuppurative panniculitis is available on the Treatment and Medication sections of the Medscape Reference Web site.
Related diseases
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
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.
References
- Panniculitis, Idiopathic Nodular. National Organization for Rare Disorders (NORD). 2007; https://www.rarediseases.org/rare-disease-information/rare-diseases/byID/297/viewAbstract. Accessed 8/30/2011.
- Panniculitis, Idiopathic Nodular. NORD. July 23, 2007; https://www.rarediseases.org/rare-disease-information/rare-diseases/byID/297/viewAbstract. Accessed 4/6/2016.
- Nodular non-suppurative panniculitis. Orphanet. March 2007; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=33577. Accessed 4/6/2016.
- Eyal Muscal. Weber-Christian Disease. Medscape Reference. Jan 22, 2015; https://emedicine.medscape.com/article/1008411-overview.
Rare Endocrinology News