Discoid Lupus Erythematosus: Causes, Symptoms and Treatment RichFeel
Discoid Lupus Erythematosus is a chronic autoimmune inflammatory disorder characterized by presence of erythematosus rashes or lesions, which are discoid in shape. It is a cutaneous condition that mainly affects the scalp, face, neck and ears. Severe form of scalp Discoid Lupus Erythematosus can cause hair loss due to Cicatricial alopecia and hyperpigmentation in the affected regions. This scalp skin condition is seen more commonly in women especially with darker skin. This article explains the clinical features of Discoid Lupus Erythematosus, causes of rashes, and available treatment options.
What Is Discoid Lupus Erythematosus?
Lupus Erythematosus refers to a group autoimmune inflammatory disorders that mainly affects the skin. However some forms can also show systemic symptoms and affect other important organs.
Discoid Lupus Erythematosus is predominantly a cutaneous condition that affects only the skin and is the most common chronic form of Cutaneous Lupus Erythematosus. The characteristic feature of this condition is the presence of discoid plaques on the scalp, face and ears. The lesions are reddish violet in color with a scaly and crusty appearance. When these rashes appear on hair bearing regions like scalp, it results in alopecia and scarring. The lesions of Discoid Lupus Erythematosus worsen on exposure to sunrays. Discoid Lupus Erythematosus often co-exists with Systemic Lupus Erythematosus that causes butterfly rash (Malar rash) on the face and may also affect the visceral organs including kidneys, blood, heart, and brain. Although Discoid Lupus Erythematosus is limited to skin, nearly 5% of people with Discoid Lupus Erythematosus develop Systemic Lupus Erythematosus.
What Causes Discoid Lupus Erythematosus?
The excat cause of Discoid Lupus Erythematosus is still not known, but it is mostly considered to be an autoimmune disorder. Autoimmunity is when the immune system of the body produces an abnormal response and attacks healthy tissues of the body. This results in an inflammation. There has been certain evidence of genetic predisposition to the disease in affected individuals. Also, certain environmental factors like stress and toxins trigger an uncommon inflammatory response leading to this disease. Sometimes a genetic basis along with a trigger by certain environmental stimuli results in this disease. It has been noted that exposure to sunrays and Ultra-Violet rays affects the keratinocytes (skin cells) and causes their destruction. Certain medications and infections have also been responsible to cause this disease.
Who Gets Discoid Lupus Erythematosus?
Discoid Lupus Erythematosus can affect both males and females of all ages, but there is a greater prevalence of the disease in females as compared to males. Children are rarely affected and the onset is after 20 years of age. Discoid Lupus Erythematosus is commonly observed in people of African-American descent who have darker skin and women of hispanic heritage. The incidence is very low in Caucasian populations with lighter skin color. Experts believe that certain genes that compromise the immune system mainly the genes known as HLA family are responsible for causing this disease. Although it is chronic skin condition, Discoid Lupus Erythematosus is not contagious.
Clinical Features of Discoid Lupus Erythematosus
The most important clinical feature of Discoid Lupus Erythematosus is the presence of characteristic scaly, discoid plaques that are destructive. The rashes appear reddish violet in color with a lighter center and deeper pigmentation towards the periphery. Primarily these rashes appear on the scalp first and then spread to other regions like face, ears, and neck. When they appear on the scalp, they manifest as crusty lesions and cause bald patches. This can lead to hair loss and pigmentation post-inflammatory response. Discoid Lupus Erythematosus is a common cause of Cicatricial Alopecia, where there is inflammation of the hair follicle and subsequent formation of crust that falls off along with the hair. This leads to hair loss along with scarring that is permanent and little can be done to promote hair re-growth. Often photosensitivity is observed in patients and the lesions aggravate on exposure to sunlight.
How is Discoid Lupus Erythematosus Diagnosed?
The diagnosis of Discoid Lupus Erythematosus requires a thorough clinical examination of the lesions and rashes. A complete medical history of the patient also helps in definite diagnosis. A skin biopsy is often done to confirm diagnosis where the investigator looks for characteristic follicular plugs, scarring and inflammation. A diagnostic test called the Lupus band test detects autoantibodies in the biopsy samples from the lesions. Distinct alopecia with scarring is also an indication of Discoid Lupus Erythematosus. Blood tests may be done to detect inflammatory markers and autoantibodies.
What is the Treatment For Discoid Lupus Erythematosus?
There is no specific cure for Discoid Lupus Erythematosus. However, there are treatments that are effective in giving symptomatic relief like confining the lesions and scarring, and preventing further development of rashes. An early diagnosis is of utmost importance as an early treatment leads to better prognosis. Treatment is mainly in the form of oral, topical, intralesional injections. Oral administration of steroids reduces inflammation and slows down the progression of the disease. Similarly, intralesional steroid injections are observed to be effective. Calcineurin inhibitors are non-steroidal topical drugs that decrease inflammation. Systemic antimalarial drugs like hydroxychloroquine have also demonstrated success in reducing the inflammation. At times, immunosuppressant like methotrexate is prescribed. Cosmetic surgeries and hair transplants are an option for individuals suffering from alopecia.
The first and the most important preventive measure is sun protection. Routine use of high SPF sunblock and broad-spectrum sunscreens offering UVA/UVB protection is recommended. Use of protective clothing against the sunrays is advised. For individuals who strictly avoid sun, are prescribed Vitamin D supplements. Another important preventive measure is to avoid smoking, as it flares the disease.
History of Discoid Lupus Erythematosus
Although Lupus is considered a modern disease, Hippocrates first studied and noted its classic symptom of red rash in around 400 BC. Lupus is Latin for ‘wolf’ and erythema is Greek for ‘redness’; which refers to the resemblance of rash to a wolf bite or to the manner in which the rash attacks like the wolf. Since its first account by Hippocrates many people have studied and documented the condition. Pierre Cazenave first used the name of the disease in 1857 and he further described Discoid Lupus Erythematosus. The disease was studied and as more information came to light, Moriz Kaposi used the terms ‘Lupus disseminated’ and ‘Lupus discoid’. Later in 1900s Sir William Osler described the systemic form of the disease. In the 20th century Lupus Erythematosus was classified to have an autoimmune basis. There were advances in the diagnostic approaches and treatment options. Over the years a lot of insight has been gained into this disease but much remains unknown.
What are the Potential Complications of Discoid Lupus Erythematosus?
The potential complication of Discoid Lupus Erythematosus is manifestation of cicatricial alopecia that results in permanent scarring and irreversible hair loss with little scope for re-growth. Sometimes, affected areas show hyperpigmentation. Another potential complication is that, about 5%of individuals suffering from this disease develop Systemic Lupus Erythematosus.
Outlook and Prognosis of Discoid Lupus Erythematosus
Discoid Lupus Erythematosus is a chronic disease with no known cure. It is known to linger for years together. But sometimes it is also observed to go into remission spontaneously. It is however a disfiguring disease causing irreversible hair loss and permanent scars. The diagnosis of the disease is a challenge as the symptoms are similar to other skin diseases such as psoriasis or eczema. An early diagnosis provides quicker treatment and better prognosis. In any case, a proper skin care routine is a good practice to confine the disease.