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What

Relapsing panniculitis is an inflammation of the fat layer beneath the skin. The affected areas of skin are usually the thighs and lower legs. These areas develop recurring nodules (bumps) that are red, round, and painful.

Who

Although relapsing panniculitis occurs most often in adults, this disease may also affect children. About 75% of all cases occur in females, with no apparent racial preference. Relapsing panniculitis is closely related to other skin conditions, and as a result, the frequency of this disease is not known.

Signs and Symptoms

In addition to the painful bumps under the skin, there are a number of other symptoms that can occur. These include fever, fatigue, nausea, vomiting, bone and joint pain, abdominal pain, and liver enlargement. Furthermore, inflammation occurs in other organs of the body occasionally, which can cause heart, lung, liver, kidney, and/or spleen problems. Although these other conditions are all possible, it is very rare for a patient to experience all of them. As mentioned earlier, the painful nodules are usually on the thighs and lower legs but have been reported on the arms, face and trunks in rare cases. Some of the skin nodules may open up and drain a yellow to brown discharge and these open sores can become infected easily.

Possible Causes

The cause of this disease has yet to be discovered and it is likely that there may be a number of potential causes that may lead to this condition, such as infections and drug side effects. Patients with this condition usually deny any thermal, mechanical or chemical trauma to the areas of affected skin. Some researchers have suggested that relapsing panniculitis is an auto-immune disease while others propose it is caused by abnormal regulation of normal inflammatory processes. This condition can sometimes occur as a result of systemic disease such as lupus erythematosus, pancreatitis, sarcoidosis or renal failure. There is a similar, more common condition known as erythema nodosum that has the same type of nodules, affects the same areas of skin and has a range of causes including certain infections and drug side effects. Some physicians believe that relapsing panniculitis is really a case of erythema nodosum in which the cause, usually an infection or reaction to a medication, goes unnoticed and therefore the symptoms continue to relapse.

Diagnosis

Skin biopsy may be required to confirm that the nodules are in fact a form of panniculitis. As mentioned earlier, this disorder is closely related to erythema nodosum and diagnosis consists of trying to determine if the cause is external, such as an infection or side effect of a medication, or internal, such as an underlying medical condition. Several lab tests can be performed to reach a diagnosis of this or another type of panniculitis depending upon the individual patient's symptoms and medical history. There are many similar disorders, and it is important to exclude these other disorders, as some of them may have different treatments.

Treatment

Treatment depends on whether or not a cause is identified. If an infection is present, antibiotics may help resolve the symptoms. However, in many cases the cause is not identified and in these cases medications that help modify the immune response are used. These include steroids like prednisone, hydroxychloriquine (Plaquenil), or azathioprine. There has recently been a case of successful use of cyclosporine, an immune suppressive medication, and a medication called clofazimine, also used to treat leprosy. Non-steroidal anti-inflammatory drugs such as ibuprofen or naprosyn can be used to reduce fever, bone and joint pain and other causes of fatigue. Antibiotic creams are encouraged if any of the nodules open in order to prevent infection.

Prognosis

Individual nodules usually disappear within a couple weeks, and may leave a small depression as a scar. Patients who experience only skin inflammation may have episodes of skin nodules that come and go, and these nodules may go into remission for many years. When Relapsing Panniculitis involves the major organ systems such as the heart, lungs, intestine, spleen, kidney, and adrenal glands, this disease can be fatal. A more accurate prediction of prognosis can be made when an underlying problem causing the nodules can be found.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Journal of Post Graduate Medicine
Short article on this disease with a helpful list of references for more information.

Panniculitis Support Group
Popular online support group.

USAToday.com
Easy-to-read information about this disorder and important questions to ask your physician.

Google Search for Relapsing Panniculitis

References and Sources

eMedicine www.emedicine.com/ped/topic2429.htm Journal of Post Graduate Medicine www.jpgmonline.com/article.asp?issn=0022-3859;year=1984;volume=30;issue=1;spage=49;epage=50;aulast=singh USAToday.com