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The medical condition known as lupus pernio actually has nothing to do with either lupus or pernio. It is a type of sarcoidosis, a disease most noted for breathing difficulties, inflammation and skin lesions. Lupus pernio is also known as Besnier-Boeck-Schaumann disease, and it is considered rare.
With this condition, areas of the skin become hard, with discoloration ranging from red to purple. Lesions form plaques, nodules or tumors, creating a disfigured appearance. The most common areas to find such lesions are the face, ears, nose and occasionally the hands or feet. These lesions are persistent, often taking weeks to respond to treatment, if they do at all.
Like chilblains or regular pernio, lupus pernio might initially be misdiagnosed as frostbite. The lesions caused by all of these conditions can be very similar, although this condition, unlike chilblains or frostbite, is not caused by exposure to cold conditions. Instead, it is thought to be a result of immune problems, much the same as other scarcoidosis. In many cases, patients who display skin lesions have also been treated for breathing or lung conditions such as scarcoidosis, problems with lymph nodes and other pulmonary diseases. These conditions are not necessarily a precursor to lupus pernio, however.
Unlike lupus erythematosus, the condition most notably known simply as lupus, lupus pernio does not present with photosensitivity. The lesions are painful if the skin breaks. Exposure to excessive sunlight can increase sensitivity and irritation, even without the photosensitivity associated with traditional Lupus.
The lesions associated with this type of sarcoidosis can leave scars or cause permanent deformities, especially concerning nasal cavities and passages. In some cases, patients respond well to corticosteroids such as prednisone or other steroid medications. The condition is recurring, however, so periodic treatment is often required and recommended.
Not much is known about the exact causes or features of the condition, beyond the belief that it is an autoimmune disease involving dysfunctional or abnormal immune responses. Statistical data indicates that most patients affected are between the ages of 20 and 40. Women are more susceptible than men are. People of Puerto Rican, Irish, Scandinavian or German decent also appear to have a higher risk for developing the condition and other forms of scarcoidosis.
Aside from skin lesions and breathing difficulties, other symptoms include fever, fatigue, weight loss and poor appetite. Many patients report feeling sick or feeling like they have the flu. Some patients report no symptoms beyond the lesions.
Like its name that is easily confused with other diseases, Lupus Pernio is often misdiagnosed as other diseases due to its wide-range of symptoms. To confuse the issue even more, the disease's presentation can range from absolutely no symptoms to severe disabilities secondary to its effects on internal organs.
Finally, Lupus Pernio is too often considered a cosmetic affliction because of the visible skin lesions associated with an outbreak. It is extremely important to recognize that the external dermal inflammatory lesions are reflected internally: in the lungs, the lymph system and even the skeletal system. Understanding that Lupus Pernio is a systemic disease may increase patient compliance with his or her physician's prescribed treatment plan.
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