Understanding Lupus Pernio: Symptoms, Causes, and Treatment
Although the name would suggest that lupus pernio is somehow a combination disease of both lupus and pernio, this condition is actually a form of sarcoidosis. Pernio involves the development of inflammation after exposure to cold temperatures, sort of like frostbite. Lupus pernio actually has nothing to do with frostbite, although it is often initially mistaken as such. Based on the symptoms that arise with lupus pernio, which are also seen in several types of lupus disease, it is understandable how the two conditions would be linked by name. That being said, sarcoidosis and lupus are completely different diseases. Keep reading to learn more about sarcoidosis, including general information about the disease, the symptoms that accompany it, how it may be caused, and treatment option that are available.
What is Sarcoidosis?
Sarcoidosis is an autoimmune disorder, meaning that the condition generally occurs as the result of one’s immune system functioning improperly. An individual who suffers from sarcoidosis likely has an overzealous immune system that reacts very strongly and inappropriately to certain triggers, such as bacteria, irritants, toxins, chemicals, or viruses. It can even be triggered by oversensitivity to environmental elements. As you may already know, sarcoidosis primarily involves the development of growths called granulomas. Granulomas are clusters of immune cells, or abnormally tissues, that can grow virtually anywhere on the body. With sarcoidosis, the most common place for these granulomas to develop is the lungs, although they may also be found in other places such as one’s skin, lymph nodes, eyes, and organs. Granulomas are non-cancerous, which is definitely good news for anyone who has been diagnosed with this condition.
Lupus pernio is a type of sarcoidosis that is considered to be very rare. It is defined by one characteristic symptom that develops in addition to other symptoms that are typical with sarcoidosis. The next section will delve into more detail about the symptoms.
Symptoms of Lupus Pernio
The most unique symptom of this type of sarcoidosis is the formation of large lesions on the skin, usually somewhere on the face although it can also occur on the hands and ears. The lesions are typically large and random in shape, although the “butterfly lesion” is common. This is the name given to a lupus-like lesion that occurs on both cheeks and the nose which imitates the appearance of a butterfly. The lesion that occurs with this form of sarcoidosis is often darkly discolored, typically shades of dark purple or red. The areas of skin affected by the lesion become hardened and raised above the surrounding skin.
The development of a large, darkly colored lesion is definitely the most characteristic symptom of this disease, but its appearance is so similar to frostbite that it is not uncommon for one’s initial diagnosis to include frostbite. The tell-tale symptom of obvious lesions on the skin only occurs in an estimated 25 percent of people with sarcoidosis. In order to make an appropriate diagnosis, there are other symptoms that need to be taken into consider. Some symptoms that are commonly associated with sarcoidosis include chest pain near the back of the sternum (the hard bone in the center of the chest), difficulty taking deep, satisfying breaths, a persistent cough, odd sound that accompanies one’s breathing, general fatigue, achy joints, general feeling of malaise, unexplained or unintentional weight loss, inflammation of old scars, rash development, headaches, dry or itchy eyes, pain inside or around the eyes, decreased vision quality, and fainting spells.
Symptoms tend to pop up between the ages of 20 to 40, but because the symptoms are also found in a number of other illnesses, sarcoidosis can go undiagnosed for a very long time.
Causes of Sarcoidosis/Lupus Pernio
Unfortunately, there are not many known causes behind sarcoidosis. In fact, scientists cannot give a 100% definite cause of sarcoidosis. There does seem to be a link between this condition and genetics. It seems that individuals who have a history of sarcoidosis in their family are more likely to develop this condition that someone who has no family history of the condition. Studies seem to suggest that those who have a family history of the condition are about five times more likely to have the disease than others without a close blood relative with the condition. Other than having a family history of the disease, scientists believe that another risk factor for developing sarcoidosis is having an overactive immune system.
The good news is that the symptoms of lupus pernio should go away on their own without any treatment, although the healing process may be slow. Corticosteroids may be prescribed to patients whose breathing, vision, nervous system, or blood pressure/heart is heavily affected by the condition. This is often the case with individuals who have a large number of lesions on the lungs, near the heart, or around the eyes. Many individuals who suffer from sarcoidosis can benefit from the use of immunosuppressive medications, that is, medicine designed to withhold the immune system so that it doesn’t react so strongly to triggers. In a worst case scenario involving severe damage to certain organs, such as the lung or liver, it may be necessary to do an organ transplant.