Lupus erythematosus diagnosis symptoms specialists

Lupus ErythematosusYou are looking for an experienced specialist in the medical field of Lupus Erythematosus? At PRIMO MEDICO you will only find specialists, clinics and centers in your field in Germany, Austria and Switzerland.

List of physicians for lupus erythematosus

Dr. med. Heidi Dotterer-Rieg

Skin and venereal diseases Frankfurt

Lupus erythematosus diagnosis symptoms specialists

Univ.-Prof. Dr. med. Stephan Grabbe

Skin and venereal diseases Mainz

Lupus erythematosus diagnosis symptoms specialists

Information about Lupus Erythematosus

What exactly is lupus erythematosus (LE)??

Lupus erythematosus belongs to a special group of chronic inflammatory diseases Autoimmune Diseases , the so-called Collagenoses .

The disease pattern is based on the body's own formation of autoantibodies against cell nucleus components. These are misprogrammed antibodies, which are directed against the body's own cells. In LE, these autoantibodies lead to inflammatory damage of the connective tie in the vascular area.

The clinical picture of LE is divided into two main groups: Systemic and cutaneous lupus erythematosus. Systemic LE is a severe disease that can theoretically affect any organ system, whereas cutaneous LE is limited to the skin. However, both forms of the disease have in common the occurrence of the characteristic butterfly erythema (butterfly lichen) localized on the face. This is a butterfly-like redness that is often seen in affected patients.

Cutaneous lupus erythematosus is further divided into four subtypes, acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), chronic cutaneous LE (CCLE), and intermittent cutaneous LE (ICLE).

Acute cutaneous LE is an acute form of cutaneous LE in which – unlike the other cutaneous forms – various organ systems may be affected. In this form of the disease, the skin changes usually occur after exposure to the sun and heal again without scarring.

Subacute cutaneous LE describes a form of progression that is regarded as an intermediate form of systemic LE and chronic cutaneous LE. This condition is characterized by both skin changes as in chronic cutaneous LE and organ involvement as in systemic LE, although the symptoms are usually milder than in systemic LE. Subacute cutaneous LE may also progress to systemic LE.

Chronic cutaneous LE comprises three further subtypes, of which chronic discoid LE is the most frequent manifestation. In addition, the lupus erythematosus profundus also includes. Chilbain lupus erythematosus belong to this subgroup.

Intermittent cutaneous LE (also called LE tumidus) is characterized by a particularly high sensitivity to light. A further form of progression in addition to the systemic. The cutaneous LE is represented by the drug-induced LE. Characteristic of this form of progression is the occurrence of lupus-like symptoms associated with the use of a drug (z.B. certain medications taken for the treatment of cardiac arrhythmias and hypertension) and spontaneously regress after discontinuation of the medication.

In Germany, women are about four times more likely to be affected by the disease than men, with the peak age between 20 and 30 years. and 40. The age of onset is between. Thus, the risk group primarily includes women of childbearing age. Drug-induced LE alone is more common in men than in women. Globally, the likelihood of disease is much higher in non-white populations than in fair-skinned populations.

Cutaneous LE is ten times more common than systemic LE.

What are the causes of lupus erythematosus??

The exact cause of the disease is largely unknown to date. However, a multifactorial genesis is thought likely, d.h. the occurrence of the disease is most likely to be caused by an interaction of various factors. These include genetic changes, hormonal factors, immune system disorders, and environmental factors such as infections or exposure to UV radiation.

Factors that specifically favor the occurrence of cutaneous LE include: Smoking, the use of specific medications (z.B. Hydrochlorothiazide) and UV exposure.

In subacute cutaneous LE, there is a correlation between disease onset and the detection of certain antigens (the human leukocyte antigens HLA B8, DR2, and DR3).

What symptoms can occur with lupus erythematosus??

General symptoms:

In systemic LE, almost all patients suffer from general symptoms such as fever, fatigue or weight loss.

Musculoskeletal system:

95% of patients are also affected by muscle pain, joint pain or joint inflammation.

Skin:

Photosensitivity, i.e. increased sensitivity to UV radiation, is present in about 2/3 of patients. This favors the occurrence of rashes. The exacerbation of pre-existing disease symptoms following UV exposure.

In 50% of the cases, the butterfly erythema already mentioned at the beginning occurs. This is a butterfly-shaped, symmetrical facial redness and skin scaling localized to the nose, forehead, and cheeks.

The skin may also show other changes. These include:

– Discoid erythema: reddish, disc-shaped areas of skin, often covered with scales and crusts – Macular exanthema: patchy skin rash – Periungual telangiectasias: visible dilatations of the superficial, small blood vessels in the area of finger and toe nails – Hair loss – Raynaud's syndrome: Paling of the fingers as a result of exposure to cold or stress, shortly thereafter the fingers typically turn blue due to a lack of oxygen, before blushing after a few minutes as a result of reactively increased blood flow – Livedo racemosa: lightning-like, bizarre-looking reddening of the skin

Nervous system

In the context of systemic LE, the nervous system can also be affected. Psychosis may occur, Headache and cognitive impairment occur.

Lung

In about 50-70% of patients, pleurisy occurs during the course of the disease, with recurrent accumulation of fluid in the area between the chest wall and the lungs ( Pleural effusion ). This often occurs in the setting of high disease activity. Pleurisy is usually accompanied by a pneumonia (lupus pneumonia) accompanies.

Heart

In some cases, systemic LE results in disease involvement of the heart. Possible manifestations include:

– Pericarditis with fluid accumulation in the pericardium (pericarditis with pericardial effusion) inflammation of the heart muscle (myocarditis)
– inflammation of the inner lining of the heart (Libman-Sacks endocarditis) – disease involvement of the cardiac vessels

kidney

In half of the cases of systemic LE, kidney involvement in the sense of lupus nephritis is present. This is an inflammatory disease of the kidney in which, depending on the severity, significant damage to kidney function can occur. Therefore, renal involvement is a major prognostic factor for the course of the disease.

Gastrointestinal tract

In addition to general symptoms such as nausea and diarrhea, inflammation of the lips (cheilitis), esophagus (esophagitis), or liver ( Hepatitis ) occur.

Eyes

Eye involvement may include conjunctivitis or severe dryness of the eye (sicca syndrome).

Pregnancy

Male and female LE patients are thought to have normal fertility rates, although studies are inconclusive.

However, pregnancy in LE patients always represents a Risk pregnancy present. Thus, there is an increased risk of premature birth, miscarriage and intrauterine (inside the uterus) growth retardation of the fetus. In addition, there is an increased risk of cardiac defects in the newborn. Therefore, patients with systemic LE. Pregnancy is not recommended in cases of high disease activity. Pregnancy should be considered only after disease activity has been successfully suppressed for at least 6 months.

In this form of progression, the following symptoms may occur:

– Inflammation of multiple joints (polyarthritis) – Pericarditis and pleurisy – Muscle pain – Rashes

Acute cutaneous LE

Acute cutaneous LE can manifest clinically in two forms.

– Localized form: This form is characterized by butterfly erythema (the butterfly lichen), which is a butterfly-shaped, little to barely scaling redness in the area of the nose, cheeks and forehead. This can either be visible only for a short time or persist for weeks. – Generalized form: Here, a blotchy, nodular rash typically appears. It can be localized on light-exposed areas, palms and soles, in the interdigital spaces between fingers and toes, on the backs of fingers or on the whole body. In addition, redness and visible vasodilatation may occur in the area of the nail folds. In the acute phase of the disease, there may also be redness, superficial lesions or ulcers in the area of the oral and nasal mucous membrane.

Subacute cutaneous LE

In this form, similar to systemic LE, general symptoms such as muscle and joint pain, feeling sick or dry eyes and mouth (sicca syndrome) are usually present.

On the skin, the following changes usually occur: Sharply demarcated, reddish, ring-shaped to oval elevations that are usually covered by a scaly layer and are reminiscent of the skin changes seen in the disease psoriasis vulgaris. After the lesions have healed, depigmented areas often remain, d.h. Skin areas that appear lighter than the healthy, surrounding skin. Only rarely, however, does scarring occur.

The skin lesions of subacute cutaneous LE occur preferentially on light-exposed areas, the extensor sides of the arms, and on the abdomen and back.

Chronic cutaneous LE

Chronic discoid LE: Here, the following characteristic skin changes occur, which are mainly localized on light-exposed areas such as the face. Only rarely do the skin changes also occur on the abdomen, back or upper arms.

– Disc-shaped, sharply defined elevations with scaling – In the center of the elevations, there is a so-called skin atrophy – Strong sensitivity to touch, which slowly spreads in a circle away from the affected area – Healing of the lesions with scarring: usually a pale scar remains in the center of the lesions, on the hairy head there may be hair loss in affected areas

LE profundus: In this form of chronic cutaneous LE, painful, reddened nodules are found that extend into the subcutaneous fatty tie and heal with scarring. They are usually localized on the buttocks, thighs and face.

Chilbain's LE: Here, prere-painful, dark red nodules appear, which are sometimes accompanied by ulceration and are mainly localized on fingers and toes.

Intermittent cutaneous LE

This variant is characterized by a particularly high sensitivity to light. Therefore, the reddish elevations and nodules typical of this form occur preferentially in areas exposed to light (face, decollete, backs of arms and upper back).

How to diagnose lupus erythematosus ?

Systemic LE

Diagnosis and initiation of therapy are often delayed due to insidious onset of disease and nonspecific symptom presentation.

If the presence of LE is suspected on the basis of clinical symptoms or medical history, a blood sample is first taken to determine the level of an antibody titer that is significant for the disease (ANA = antinuclear antibodies). In affected patients, this usually exceeds a certain threshold and is considered the first indication of the presence of the disease.

In a comprehensive analysis, the blood sample is also examined for the presence of other parameters and antibodies that are typically altered in the case of the disease. There are several antibodies that are clustered in affected patients. In addition, patients with the disease show further changes in the blood count: Sufferers often have a deficiency of white and red blood cells and suffer from anemia. In addition, there are often elevated inflammation parameters. In the case of renal involvement, elevated renal values before.

In the next step, the patient's individual symptoms are recorded using predefined classification criteria. On the basis of the symptoms present in each case, further necessary examinations are carried out, which are then arranged with a specialist.

To test for photosensitivity, photoprovocation with UV light is usually performed, d.h. Skin is irradiated with UV light. The skin reaction observed. Because of the skin's sensitivity to UV light, UV exposure typically causes reddening of the skin in patients with LE.

Furthermore, a skin biopsy is often performed. Here, a small piece of skin is removed. Examined microscopically. Thus, the antibody deposits typical of the disease can be. Inflammatory changes in the skin are detected.

If there is a clinical suspicion of kidney involvement (so-called lupus nephritis), a kidney biopsy (removal of a small piece of kidney tie) is performed for clarification.

In some cases, where there is a concrete suspicion of the presence of pleurisy with pleural effusion, a puncture of this effusion fluid is performed. The fluid can then be examined in the laboratory.

In the presence of this form of progression, typical antibody constellations are found in the blood test.

This form can usually be distinguished diagnostically from systemic LE by differences in the skin biopsy.

In some cases, the individual subtypes of cutaneous LE also show different antibody constellations in the blood test. How is the treatment carried out?. Therapy of lupus erythematosus?All patients with LE should apply a sunscreen ointment regularly and avoid direct sun exposure.

Systemic LE

In addition, systemic LE can be treated with medication to alleviate the symptoms and reduce the number of phases with increased disease activity (exacerbations).

In mild courses without involvement of vital organs, pain and anti-inflammatory substances (so-called NSAIDs = non-steroidal anti-inflammatory drugs) and anti-malarial drugs (hydroxychloroquine) are used for this purpose. In the case of inflammatory relapses, glucocorticoids (cortisone variants) are also included in the therapy regimen.

In the case of a severe course with involvement of vital organs, glucocorticoids are usually used for treatment. In some cases, these are combined with an immunosuppressive drug (usually azathioprine, alternatively methotrexate or other immunosuppressants).

In the presence of renal involvement (lupus nephritis), glucocorticoids and immunosuppressants are also used. In addition, further measures can be taken to protect renal function.

In cutaneous LE, topical therapy is usually performed, d.h. a therapy in which the medication is applied locally to the affected areas of the skin rather than taken as tablets. Glucocorticoids are usually used here. Alternatively, the immunosuppressant tacrolism, vitamin D-related preparations, and antibiotics (macrolides) may be used. In the case of a very severe course, an antimalarial drug (hydroxychloroquine) must be taken in addition to local therapy.

What are the prognosis and chances of cure for lupus erythematosus??

Systemic LE

The disease can be acute or subacute. However, the most common course is chronic relapsing LE, with years of remission (periods without any disease activity or symptoms) between each relapse. Exposure to sunlight, infections, surgeries and pregnancy can trigger the individual relapses. After menopause, the episodes usually occur less frequently in women.

Living in countries with high levels of development ca. 95% of patients live at least 10 years after diagnosis. However, the exact prognosis varies from individual to individual. Varies depending on the course of the disease. In particular, low inflammatory activity has a favorable effect on prognosis. Early diagnosis and treatment of kidney damage is also beneficial, as it can prevent severe kidney disease. Furthermore, lupus patients have an increased risk of heart disease.

Drug-induced LE

After discontinuation of the medication, the symptoms of the disease usually disappear spontaneously.

Cutaneous LE

Cutaneous LE usually shows a chronic course with alternation of improvement and relapses. In 5% of cases, cutaneous LE may progress to systemic LE, although this is extremely rare in the common subtype of chronic discoid LE.

Intermittent cutaneous LE also usually has a very good prognosis; in some cases, the symptoms of the disease even regress spontaneously.

Which physicians and clinics can diagnose and treat lupus erythematosus??

Diagnosis and treatment of lupus erythematosus are usually carried out by an interdisciplinary team consisting of physicians from different specialties.

Rheumatologists are the specialists who are primarily responsible for the diagnosis and treatment of lupus erythematosus as a rheumatological disease. In the case of organ involvement, other specialists should be consulted, depending on the organ systems affected. If symptoms are noticeable, a resident internist can perform initial tests. Refer the patient to the appropriate specialists.

Those who need a doctor want the best medical care for themselves. That is why the patient asks himself, where can I find the best clinic for me?? Since this question cannot be answered objectively and a reputable doctor would never claim to be the best doctor, one can only rely on the experience of a doctor.

We help you find an expert for your disease. All listed physicians and clinics have been reviewed by us for their outstanding specialization in the field of Lupus Erythematosus and are awaiting your inquiry or request for treatment.

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