Discoid Lupus Erythematosus

DEFINITION

Discoid Lupus Erythematosus is a skin disease that causes scaling and redness of the skin lesions are exacerbated by exposure to sunlight. Red spots on the skin usually in the form of coins. The most important place for the LED lesions usually occur on the face, neck, forehead, ears, chest, shoulders and upper back. Lesions of the central part is usually brightly colored than the lesion edges darker than normal skin.


Discoid Lupus Erythematosus is closely related to another condition called Systemic Lupus Erythematosus [LES]. LES is an autoimmune disease, which means the defense system [immune] makes the body's natural cells and tissues as a target and destroy it. Lupus erythematosus refers to disorders that affect broad. In LES abnormalities can affect internal organs, while the LED only affects the skin, but there is a small LED that attack internal organs, which makes the patient became ill.

Discoid Lupus Erythematosus is always limited to the skin, but the LES also affect the joints, kidneys, heart, liver, and blood in addition to the skin. About 10% of people affected with discoid lupus erythematosus can head to the LES. Impossible to predict anyone who can both suffer from the LES, and it is impossible to prevent the onset of LES. Some experts believe that people from the LED to the LES LES may be a patient with discoid lesions as the main complaint. When people are diagnosed with LES, it is important to do regular follow-ups with your doctor so that the doctor can both monitor the progress of the condition and recognize the symptoms of LEDs to LES.



Epidemiology

In the world, the prevalence of LES ranges from 17-48 cases per 100,000. The highest prevalence occurs in people aged 40-60 years. Typically 2-3 times more common in women than men. LED ranges from 50-85% of cases of lupus erythematosus kuntaneus. Patients with rare LED kalihatan systemic disease clinically. Lesions may arise as scarring or atrophy. Alopecia lesions disrupt the dross. LEDs usually attacks on America and race arfika rare in Caucasian and Asian. LEDs can occur in all ages but especially at the age of 20-40 years, with an average age of 38 years. LED is also ranging between 15-30% of the population LES cases. 5% of cases can lead to LES LEDs.



Etiology

Discoid Lupus Erythematosus is an autoimmune disease thought to be. Autoimmune disorders occur when the immune cells attack the body's own misguided. Normally, immune cells recognize and destroy invasion work outside, such as bacteria, viruses, and fungi. The incidence increased in those with high HLA combinations. Usually triggered by sunlight. The exact cause of the LED is not yet known. Experts believe that a combination of genetic, environmental and hormonal factors are involved in the formation of the LED. Since there is no specific gene for LEDs are found, the researchers talah found several genes that contribute to the formation of this disease. And some people who have this gene increases the risk of the formation of the LED. The disease can also be induced by drugs, such as procainamide, hydantoin, griseufulvin, phenyl butazone, penicillin, streptomycin, tetracycline, and sulfonamide and referred to as SLE like syndrome.

Exposure to sunlight [ultraviolet radiation] plays an important role in some cases of LEDs. Most LEDs rush occurs in areas exposed to direct sunlight. Exposure to sunlight can trigger the formation of a new rush. In some people, the disease disappears during the winter, where there is little sun. Psychological stress and viral or bacterial infection of the skin also trigger LED Dapa. LED tidal contagious, the disease is not transmitted by skin contact or changing personal items such as towels, comb or razor.



Pathogenesis

Discoid lupus erythematosus begins with somatic mutations in cell lymphocytic origin [lymphositic stem cells] in people who have a predisposition. Genetic factors do exist. In LEDs, the immune cells that attack is believed to be one of the types of white blood cells [WBCs], namely T lymphocytes in the skin lesions and scarring is the result and the process of inflammation and lesions characteristic form diskiod.

An increase in HLA-B7, -B8, -DR2, -DR3 and - DQA0102 and decreased HLA-A2 has been reported in patients with Discoid Lupus Eritematoses. The combination of HLA-DR3, HLA-B7 and HLA-DQA0102 estimated maximum risk for the LED. LEDs also increased among women with careers of X-linked chronic granulomatous disease. In patients LED both men and women aged 15-39 years, there is an increased incidence of HLA-B7, and women over 40 years, there is an increased incidence of HLA-B8.

Pathogenesis is also allegedly associated with the immune system and autoimmune disorders that occurred associated with genetic individuals, which is an autoimmune disorder occurs when the immune cells to recognize the antigens so rnenyerang one's own body. Normally, immune cells work to identify and help attack foreign bodies such as bacteria, viruses and fungi that enter the body, but in the presence of immune system disorders, the immune cells to recognize one of the body's tissues are considered as foreign and will then attack and destroy the tissue. Interleukin [IL] -1 receptor antagonist and tumor necrosis factor [TNF-α] polymorphic genes have been referred to as the genetic factors of LE. Found an increased prevalence of promoter polymorphism of TNF-α [308A] in patients with LE.



CLINICAL SYMPTOMS

In Eritomatosus Discoid lupus, skin lesions are round and raised. Rush redness, measuring 5-10 mm in the central part biasanra brighter than the edges. The surface of the lesion is usually 'warty'. Usually there is no itching or pain associated with the lesion. It usually occurs on the face, ears, neck, forehead, chest, back, and arms .. As usual lesions increase in size, these lesions can be thinned and widened. Without treatment, the lesion boundaries widened gradually phase out the central part of the mongering [brightened] and getting thinner causes scarring. The lesions are rarely painful and rarely itch. When the LEDs healed lesions, lesions that leave a thin layer on the skin.

Some LEDs will perform in the face in a butterfly-shaped lesion [butterfly erythema] that cover the cheeks and nose. These lesions lead to scarring caused small but spread the thickened capillary commonly occurs on the face. Lesions can occur on the lips and in the mouth. If the lesion occurs on the forehead, then it can lead to the collapse of the hair follicle and result in bald areas permanently. People with LEDs usually sensitive to sunlight. Their skin often looks like a sunburn and sun often aggravate their condition.

This disease can leave sikatrik artrofik, sometimes hypertrophic, and even distortion of the ear or nose. The nose can be shaped like the beak of a cockatoo. Part of the body that are not covered by clothing, which is exposed to direct sunlight beresidif faster than other parts. Lesions can occur dimukosa, namely dimukosa oral and vulva, or in the conjunctiva. Clinical appears desquamation, ulceration and sometimes sikatrisasi.

Clinical variants of LED:

1. Lupus Ertitematosus Tumidis, erythematous patches of brown elevated look upfront, knees and heels. The clinical picture may resemble erysipelas or cellulitis.

2. Lupus Erythematosus Profunda, nodes in the layout, look on the forehead, neck, buttocks, and upper arms. The skin over the node erythematosus, atrophic, or ulcerated

Lupus pernio [chilblain lupus, Hutchinson], the disease consists of patches of erythematous infiltrated in the areas not covered by clothing, deteriorated in the cold.



Examination support

1. Serologic tests

- Some patients with LEDs [about 20%] manifest in antinuclear antibody positive when tested.

- Anti-Ro [SS-A] autoantibodies present in 1-3% of patients

- Antinative DNA or anti-Sm antibodies usually describes the LES and occurs in patients beberpa

2. Findings of other Laboratoium

- Cytopenias can terjad

- Erythrocyte sedimentation rate in some patients

- Rheumatoid factor positive Dapa

- Urinalysis tapat describe the presence of proteinuria in renal output

Other tests

1. Immunopatologi

- Deposit immunology and complement the dermal-epidermal a characteristic appearance. The network was tested were taken from skin lesions or normal.biopsi normal tissue can be taken from the surface of the exposed or unexposed. Tests for non-exposed skin lesions are non Lupus Band Test [LBT]

- The use and interpretation of these tests is based on the biopsy. Approximately 90% of patients with manifestations of LEDs leads to test immunoflourence [DIF] berlesi skin. Membrane area of the skin lesions are not specific for lupus and other skin diseases can be. Lesions were old or very new can be interpreted negatively on the picture immunoflourence microscopy.

If the skin biopsy has led to Discoid lupus erythematosus should be performed other tests such as blood tests.


DIAGNOSIS

Diagnosis can be established based on a combination of history, physical examination and investigations. The presence of plaque in the area demarcated lesions include:

     Erythema and telengiektasis
     Scales [scale]
     follicular plugging
     Pigmentary changes [clearer in color] including central hypopigmentation and hyperpigmentation lesions of the peripheral areas of lesions
     Scarring alopecia and, if the lesion is in the region of the scalp
     When the lesions on the nose of the cheek berkonfluensi, can shaped like a butterfly [butterfly erythema]

The diagnosis of discoid lupus erythematosus typically requires a skin biopsy. A biopsy is used to confirm the diagnosis. Examples of lesions taken with further special preparation was observed under mikroskop.Tes blood can not explain the type of antibody that of the LEDs and the appearance of scales typically do not provide any explanation other skin lesions. Usually the lesions that have characteristics of a lesion can be identified for the LED. If there are antibodies in the blood or a symptom of other physical signs, leading to a likely diagnosis LED. Direct Immunoflorescencemenunjukkan deposits of IgG, IgM, IgA, and C3 in the basement membrane. Blood screening tests for the diagnosis of SLE is also recommended.



DIAGNOSIS

Diagnosis of LEDs include:

1. Dermatomyositis.

Is an autoimmune disease that attacks the muscle and skin. Vaslkulitis and kalsinosis a slow symptoms in children, in adults something to do and systemic malignancy. their purplish skin periorbital edema, dorsum, and linear erythema on the dorsum of the phalanges.

2. Erythema multiforme

The classic lesion is a lesion that is shaped like a slice or a target. Erythema were round or oval with a purplish color centers. Typical distribution on the extensor surfaces of the arms and legs, but it is an important diagnostic is found on the palms of the hands and feet.

3. Lichen Planus

Lichen planus is a disorder rather vary in shape and form most often is itchy papules. Predilection in the wrists, feet and back. The surface is flat and looks like a delicate woven of spots and stripes as Wickham's striae, shiny and polygonal,

4. Psoriasis

Erythematous macules that amount varies from billion to numular, this chronic recurrent disease with scaling. Lesions of the shiny white. The presence of scale which, when scratched shows signs drip wax, scratches forwarded, arising auspitz with blood spots and Koebner phenomenon.

5. Systemic Lupus Erythematosus [LES]

In LES mucosal lesions in more often, konstusional symptoms such as fatigue, fever, weight loss is more common. Laboratory and immunological abnormalities are also common. LES is attacking systemic organs, such as found in:

- The kidney is about 68% of proteinuria, hematuria, and nephrotic syndrome.

- Cardiovascular form of pericarditis and pericardial effusion.

- Lungs pleural effusion and pneumonitis.

- Gastrointestinal tract, abdominal pain and may be accompanied by nausea, vomiting, diarrhea.



MANAGEMENT

a. prevention

The purpose of the LED therapy is to improve the quality of life of patients, control of existing lesions, reducing the former lesions, and to prevent further progression of lesions.

Treatment begins with avoiding trigger factors eg heat, medicine and of course, sunshine and all the sources that cause exposure to UV radiation. The means used to protect the skin is to wear clothes that covered, wide hat. In addition, patients are advised to avoid the use of drugs such photosensitive Hidroclorothiazid, tetrasklin, griseofulvin, and piroxicam. [11,16]

b. Topical therapy

     Sun protection using a broad-spectrum sunscreen waterproof [≥ SPF 15 with UVA inhibiting agents such as titanium dioxide parsol and mikronized.
     Glucocorticoids locally. Although the potential use of the medium of this preparation as triamcinolon acetonide 0.1% in the sensitive areas of the face, such as topical medications superpoten kelassatu proprionat clobetasol or betamethasone diproprionate give satisfactory results on the skin. Use of 2 times a day for 2 weeks followed by 2 weeks rest period to minimize complications such as atrophy and telengiektasis. The ointment is more effective than the cream in hyperkeratotic lesions.
     Intralesional glucocorticoids. Use intalesi glucocorticoid triamcinolone acetonide suspension as 2.5 to 5 mg / ml in the face with the highest concentration allowed in the skin less sensitive. It is indicated for hyperkeratotic lesions or lesions that do not respond to local corticosteroid use, however, patients with lesions that are too much needs to be careful with the use of this therapy.

C. Systemic Therapy

Anti-malaria drug is an effective option for the LED. Chloroquine [CQ] Hidroklorokuin [HCQ], and kuinakrin are three commonly used drugs. The mechanism of this drug is

     Interfere in the process of antigen in macrophages and other antigen presenting cells
     Reduce the formation of peptide - Major Histocompatibility Complex [MHC] complex proteins that decrease the stimulation of autoreactive CD4 + T cells and decreases the release of cytokines.
     Introducing apoptosis in lymphocytes, and
     Lower levels of IL-6, IL-1α, and TNF-α ..

In some patients, hidoklorokuin started at a dose of 200 mg per day to assess tolerance to the gastrointestinal tract administered dose. If the patient does not have diarrhea or gastrointestinal disorders increased the dose was doubled to 200 mg twice per day. Maximum dose of less than 6.5 hidroklorokui mg / kg / day. Giving hidroklorokuin during 3-4minggu first then the dosage is reduced slowly over 3-4 weeks later by administration of 1 times a day. While Kuinakrin may be granted if there is no response to chloroquine and hidroklorokuin. Side effects of chloroquine is retinopathy in eyes, headache and drowsiness gastrointestinal system disorders.

Pharmacotherapy aims to reduce morbidity and to prevent complications. Hidroksikloroquin and Chloroquine has shown beneficial results in treating LEDs. Alternative therapies, anecdotal reports and small trials found the following drugs may be useful in some patients: dapsone, auranofin, quinakrine, thalidomide, isotretinoin, acitretin, azathioprine, mycophenolate mofetil, phenytoin, interferon, and monoclonal antibodies simerik.

Drug Category: Anti-Malaria Drugs - may have immunomodulatory section. Hidroksikloroquin is the drug of choice [drug of choice] when systemic medications needed for the LEDs. Chloroquine is the drug of second choice.