The medical condition known as lupus pernio has nothing to do with lupus or pernio. It is a type of sarcoidosis, a disease best known 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 are hardened, with discoloration ranging from red to purple. The lesions form plaques, nodules, or tumors, creating a disfigured appearance. The most common areas to find such injuries are the face, ears, nose, and occasionally the hands or feet. These lesions are persistent, often taking weeks to respond to treatment, if at all.
Like chilblains or regular pernio, lupus pernio may initially be diagnosed as frostbite. The lesions caused by all of these conditions can be very similar, although lupus pernio, unlike chilblains or frostbite, is not caused by exposure to cold. Instead, it is believed to be the result of immune problems, like other scarcoidoses. In many cases, patients with skin lesions have also been treated for respiratory or lung problems, such as scarcoidosis, lymph node problems, and other lung diseases. However, these conditions are not necessarily a precursor to lupus pernio.
Unlike lupus erythematosus, the condition better known simply as lupus, lupus pernio is not photosensitive. The lesions are painful if the skin is broken. 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 in relation to the nasal cavities and passages. In some cases, patients respond well to corticosteroids such as prednisone or other steroid medications. However, the condition is recurrent, so regular treatment is often required and recommended.
Not much is known about the exact causes or characteristics of lupus pernio, beyond the belief that it is an autoimmune disease that involves dysfunctional or abnormal immune responses. Statistical data indicate that the majority of affected patients are between 20 and 40 years old. Women are more susceptible than men. People of Puerto Rican, Irish, Scandinavian, or German descent also appear to be at increased risk of developing lupus pernio and other forms of scarcoidosis.
In addition to skin lesions and breathing difficulties, other symptoms include fever, fatigue, weight loss, and poor appetite. Many patients report that they feel sick or have the flu. Some patients report no symptoms beyond the lesions.