Introduction:
Lupus erythematosus panniculitis (LEP) is an autoimmune disorder that is quite rare in incidence. It is yet another clinical variant of lupus and is said to affect the panniculus.
Panniculus refers to the subcutaneous layer of fat that is present just below the skin. This atypical disease is characterized by the development of small, erythematous nodules over the skin, particularly involving the face, breast, thighs, and buttocks. The disease is highly prevalent in women compared to men. LPE is generally found to occur alongside a co-existing disease such as discoid lupus erythematosus (DLE) or systemic lupus erythematosus (SLE). However, its incidence may also be reported in an isolated manner. This article aims to dive deeper into the pathophysiology of this uncommon disease and provide a general overview of the management of LEP.
What Are the Symptoms of Lupus Erythematosus Panniculitis?
The characteristic hallmark of this disease is the formation of painful, tender nodules under the skin. These nodules are about 0.39 to 1.97 inches in dimension and do not resolve in a short time. LEP disease is an inflammatory response observed in the subcutaneous fat. The nodules may transition to much-advanced skin lesions with chronic and persistent inflammation. It is relatively much easier to spot these nodules on white-skinned people compared to people with darker tones.
What Causes Lupus Erythematosus Panniculitis?
Lupus is categorized as an autoimmune disease. The exact cause of lupus and its variants is not certain to date. Autoimmune diseases are recognized as conditions triggered by one’s immune system itself. The body has a defense system that is capable of warding off any foreign invasion or harmful organisms like bacteria and viruses. However, in some select cases, the immune system is highly activated, resulting in an abnormal immune response. The immune system mistakenly attacks the body’s healthy tissues by producing inflammatory chemicals called chemokines. In panniculitis disease, the healthy tissue of the fat layer present below the skin is targeted, which leads to inflammation of the panniculus. Over time, symptoms are noticeable.
Women tend to show a higher rate of occurrence of autoimmune diseases owing to their highly active immune system during childbearing age. Multiple theories have been proposed to explain autoimmunity. The three widely accepted reasons are genetic makeup or family history, environmental factors, and immunological factors.
Who Is Affected by Lupus Erythematosus Panniculitis?
Lupus erythematosus panniculitis is a rare disease that is usually found to occur alongside another variant of lupus, such as discoid lupus erythematosus or systemic lupus erythematosus.
Anyone can acquire this disease. However, middle-aged women are most prone to suffer from its incidence. Certain factors that may trigger the disease include genetics, exposure to UV (ultraviolet) rays, side effects of certain medications, a habit of smoking, and some viruses.
How Is Lupus Erythematosus Panniculitis Diagnosed?
This lupus variant may pose a challenge to make an accurate diagnosis owing to the resemblance of its symptoms to other variants and conditions like T-cell lymphomas. A skin biopsy is the most reliable diagnostic approach to make a confirmed diagnosis of LEP. One should make sure to choose an appropriate laboratory for carrying out the tests. A well-established communication between the clinician and the laboratory practitioner is essential for minimal diagnostic error.
How to Treat Lupus Erythematosus Panniculitis?
Lupus erythematosus panniculitis, once diagnosed, must receive immediate treatment. Untimely treatment may prove to have serious consequences that may also involve other vital organs of the body like kidneys and heart. Upon noticing visible skin changes, one must avoid further delay and contact the healthcare provider for a thorough diagnosis.
The first line of treatment administered to patients suffering from LEP is steroids. These are taken either orally, applied topically, or injected directly into the panniculitis lesions. The second commonly administered drug includes antimalarial drugs like chloroquine or plaquenil. The disease flare-ups are observed to be manageable with a treatment plan involving these two therapies. However, remission of the disease is quite a possibility as there is no definite cure for lupus found to date.
The inflammatory lesions associated with panniculitis are quite painful and may lead to evident tissue loss and scarring. The nodules develop in areas involving the face and upper and lower extremities. This can lead to a significant loss of self-esteem in LEP patients and have a deteriorating effect on their mental well-being. The psychological impact caused by LEP also needs to be addressed when considering the treatment plan for panniculitis disease. Consulting a mental health therapist is a good start to overcoming this aspect of the disease. Apart from this, the skin damage and tissue loss can be compensated for with the help of dermal filler injections. However, the doctor’s approval for initiating such treatment needs to be obtained as people with autoimmune disorders are highly unlikely to show a good prognosis for filler treatments.
Conclusion:
Lupus is a common autoimmune disorder that has several variants. Lupus erythematosus panniculitis is one such variant. However, its incidence is quite rare. The LEP disease is manageable with symptomatic treatment. One can live a life as good as normal, provided appropriate steps are taken to control disease flare-ups and prevent remission. Getting a treatment becomes important for lupus patients. Being negligent of the condition can lead to serious complications that may prove to be life-threatening. The renal and cardiovascular systems are highly affected in patients with untreated lupus. The research for a definite cure for lupus is still in the works. However, medications and lifestyle modifications have proved to be well enough for managing the disease.
