Dubois lupus erythematosus and related syndromes ninth. Systemic lupus erythematosus sle, is the most common type of lupus. American college of rheumatology classification criteria for. Cutaneous lupus erythematosus le may present in a variety of clinical forms. Three recognized subtypes of cutaneous le are acute cutaneous le. The disease has been studied in dogs as the animal model for sle in humans. Cutaneous lupus erythematosus cle is a heterogeneous autoimmune skin disease that can present with or without systemic lupus erythematosus sle.
The 2019 eularacr classification criteria for sle include positive ana at least once as. Approximately twothirds of people with lupus will develop some type of skin disease, called cutaneous lupus erythematosus. Pdf cutaneous lupus erythematosus issues in diagnosis. Skin disease in lupus can cause rashes or sores lesions, most of which will appear on sunexposed areas such as the face, ears, neck, arms, and legs. Cutaneous lupus erythematosus 3 categories of lespecific skin diseases 1. Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs. The underlying cause of le is unknown but the etiology is thought to be multifactorial. The rash of acute cutaneous lupus erythematosus can be transient and heal without scarring, although persistently active rashes may result in. The word lupus is derived from the latin word for wolf and it refers to the characteristic butterflylike rash on the face, which is similar to the white markings on a wolfs face. Lupus erythematosus le is a connective tissue disease and autoimmune disorder that can affect one or several organs.
Disease severity is wide ranging, with most suffering milder. Lupus is the latin word for wolf and has been used to name various skin diseases at least since the 10 th century. From basic scientific principles to practical points of clinical management, the updated 9th edition provides extensive, authoritative coverage of systemic lupus. Lupus, technically known as systemic lupus erythematosus sle, is an autoimmune disease in which the bodys immune system mistakenly attacks healthy tissue in many parts of the body.
Systemic lupus erythematosus, lahita rg ed, churchill livingstone, new york 1992. Lupus was fi rst recognised as a systemic disease with visceral manifestations by moriz kaposi 18371902. Systemic means that it affects many organs of the body. Dubois lupus erythematosus and related syndromes ninth edition. People of all races and ethnic groups can develop lupus. Symptoms vary between people and may be mild to severe. There are over 100 medications that are known to cause lupuslike side effects in certain people. Sometimes lupus only affects the skin as in chronic cutaneous or discoid lupus. Managing cle is important in order to reduce potential and established areas of damage, as well as improve quality of life qol.
Cutaneous manifestations of systemic lupus erythematosus. Circulating autoantibodies and immune complexes are due to loss of normal immune tolerance and are pathogenic. Systemic lupus erythematosus sle is an autoimmune disease with a prevalence of 36. Cutaneous lupus erythematosus images codes and concepts open go to the image library. The aim of this chapter, which is set out as below, is to highlight the key features of these conditions. Subacute cutaneous lupus erythematosus scle differential. Lupus erythematosus lupus erythematosus le may be seen in one of two wellrecognized forms systemic acute lupus erythematosus sle profound impact it has on many organs discoid chronic lupus erythematosus dle chronic and localised skin lesions involving the bridge of. Subacute cutaneous lupus erythematosus scle workup.
If you have problems viewing pdf files, download the latest version of adobe reader for language access assistance, contact the ncats public information officer genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Although the term is frequently used alone to designate lupus vulgaris and sometimes lupus erythematosus, without a modifier it has no meaning. Jun 19, 2015 systemic lupus erythematosus sle is an autoimmune disease with a prevalence of 36. Cutaneous manifestations are frequently the presenting sign of le, and in the case of certain cutaneous le cle subtypes, they can occur in the absence of systemic disease. Click on lupus foundation of america to view the information page. Although both men and women of all age groups can be affected, women outnumber men almost 10 fold and. This is a pdf file of an unedited manuscript that has. The diagnosis and treatment of systemic lupus erythematosus. All forms of skin rashes in lupus are made worse by exposure to light particularly sunlight. Scle is highly photosensitive, with 7090% of patients meeting the acr definition of abnormal photosensitivity.
Lessons from practice lower lip ulceration and thickening is commonly caused by lupus erythematosus in northern australia there is a high incidence of both systemic and cutaneous lupus erythematosus in indigenous australians. Lupus erythematosus le is a chronic, autoimmune disease that includes a broad spectrum of symptoms. Women of color are 23 times more likely to develop lupus. Pdf cutaneous lupus erythematosus issues in diagnosis and. Cutaneous lupus erythematosus clinical and laboratory. Clinical characteristics of each group are unique 8. Cutaneous lupus erythematosus lupus is an autoimmune disease, which affects multiple organs and systems in the body. The symptoms of this form of lupus include a rash on the face, scalp, or elsewhere.
With regards to the skin, there are lupus specific skin lesions and nonspecific skin lesions. Subacute cutaneous lupus erythematosus is a clinically distinct subset of cases of lupus erythematosus that is most often present in white women aged 15 to 40, consisting of skin lesions that are scaly and evolve as polycyclic annular lesions or plaques similar to those of plaque psoriasis. Guideline on clinical investigation of medicinal products for the. Cutaneous lupus erythematosus cle is lupus affecting the skin. Nondrug therapy should be used in every case, which includes photoprotection, smoking cessation, and avoiding drugs. The 10 year survival rate is exceeding 85% according to recent reports. Systemic lupus erythematosus sle is a progressive autoimmune disease that results in inflammation and tissue damage characterized by flares, spontaneous remission, and relapses, sle is a chronic disease. Dubois lupus erythematosus and related syndromes has been recognized internationally as the goto clinical reference on lupus and other connective tissue diseases. Th e systemic form was further established by osler in baltimore and jadassohn in vienna. Nondrug therapy should be used in every case, which includes photoprotection, smoking cessation, and. It has been considered the great imitator of many different diseases. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli. Oct 11, 2016 cutaneous lupus erythematosus 3 categories of lespecific skin diseases 1. However, men, children, and teenagers develop lupus, too.
Systemic lupus erythematosus is a systemic autoimmune disease with a worldwide distribution. It is important to distinguish cutaneous lupus from systemic lupus erythematosus sle. Cutaneous lupus erythematosus genetic and rare diseases. Cutaneous lupus erythematosus definition of cutaneous lupus. Pdf cutaneous lupus erythematosus le may present in a variety of clinical forms.
Subacute cutaneous lupus erythematosus scle is a nonscarring, nonatrophyproducing, photosensitive dermatosis. Chronic cutaneous lupus erythematosus ccle is more common in women, affecting from 1. Lupus erythematosus le is a chronic, autoimmune, multisystem disease that displays many diverse symptoms in which localized cutaneous le cle is on one end of the spectrum and severe systemic le sle on the other end. Sontheimer2 1 private dermatology practice, coralville, iowa, usa 2 department of dermatology, university of utah school of medicine, salt lake city, utah, usa. Lupus caused by medication is known as druginduced lupus. Acle may be localized most often as a malar or butterfly rash or generalized. Lupus strikes 1 in 400 white woman and 1 in 250 black women, it is also common in china. Cutaneous lupus erythematosus issues in diagnosis and treatment hobart w.
Systemic lupus erythematosus sle is a progressive autoimmune disease that results in inflammation and tissue damage characterized by flares, spontaneous remission, and relapses, sle is a chronic disease sle can affect any part of the body but often results in. Lupus information sheet continued lupus strikes mostly women of childbearing age 1544. Sle is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs. Lupus erythematosus le comprises an uncommon group of connective tissue disorders. Systemic lupus erythematosus sle is a complex autoim. Chronic cutaneous lu pus erythematosus comprises discoid lupus erythematosus dle and lupus profundus lp. Cutaneous lupus erythematosus encompasses a wide range of dermatologic. Cle is thought to be an autoimmune condition where specific antibodies are produced and cause damage to the skin resulting in the appearance of a rash. Cutaneous lupus refers to a form of the disease in which symptoms are restricted to those that affect the skin. Systemic lupus erythematosus combines basic science with clinical science to provide a translational treatment of the disease and is a useful reference for specialists in the diagnosis and management of patients with sle, a tool for measurement of clinical activity for pharmaceutical development and basic research of the disease and a reference.
It can affect the joints, skin, brain, lungs, kidneys, and blood vessels. Systemic lupus erythematosus sle sle is a chronic autoimmune disease that can a. The american college of rheumatology acr established 11 criteria as a classificatory instrument to operationalise the definition of sle in clinical trials. The rashes seen in lupus can be specific in shape and pattern. Apoptosis in chronic cutaneous lupus erythematosus, discoid lupus. The name systemic lupus erythematosus dates back to the early 20th century. Subacute cutaneous lupus erythematosus is another lupusspecific rash. Acute cutaneous lupus erythematosus acle may occur as a localized, occasionally transient, or generalized widespread form. The recognition and management of discle can be challenging, as the condition may be triggered by different classes of drugs after variable lengths of time. Cutaneous lupus erythematosus is categorized as acute, subacute, and chronic. Scle commonly develops in sunexposed areas, including the upper back, shoulders, extensor arms, neck, and upper torso, while the face is often spared.
Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. Lupus erythematosus primary care dermatology society uk. Systemic lupus systemic lupus erythematosus a multisystem autoimmune disease etiology unknown most common in women, young to most common in women, young to middleaged highly variable clinical presentation highly variable clinical presentation in individual patients harvardmit division of health sciences and technology. Lupus, cutaneous cle and systemic lupus erythematosus sle. The clasi cutaneous lupus erythematosus disease area and. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli cated and challenging disease to diagnose and treat. Subacute cutaneous lupus erythematosus observed by a clinician. Systemic lupus erythematosus diagnosis and treatment. Cutaneous lupus erythematosus piell latinoamericana. The skin is one of the target organs most variably affected by the disease. Systemic lupus erythematosus, referred to as sle or lupus, is a chronic longterm disease that causes systemic inflammation which affects multiple organs. Autoimmune disease, subacute cutaneous lupus erythematosus, discoid lupus erythematosus, acute cutaneous lupus erythematosus, sun protection, antimalarials, retinoids, thalidomide, corticosteroids definitiondiagnostic criteria lupus erythematosus le is a chronic. Types cutaneous lupus only affects the skin neonatal lupus affects newborn babies druginduced lupus resolves when offending drug is stopped systemic lupus erythematosus sle can affect any part of the body.
It is a common skin manifestation in druginduced lupus. As with sle, subacute cutaneous lupus erythematosus scle occurs primarily in young to middle aged women. Three recognized subtypes of cutaneous le are acute cutaneous le acle, subacute cutaneous le scle, and chronic cutaneous le ccle. When healthy, our immune system protects the body from germs and cancers. Autoimmune disease, subacute cutaneous lupus erythematosus, discoid lupus erythematosus, acute cutaneous lupus erythematosus, sun protection, antimalarials, retinoids, thalidomide, corticosteroids definitiondiagnostic criteria lupus erythematosus le is a chronic, autoimmune, inflammatory disease that presents. Lupus erythematosus, chronic cutaneous, lupus erythematosus, discoid, lupus erythematosus, cutaneous, chronic, dle, lupus erythematosus discoid, dle discoid lupus erythemat, lediscoid lupus erythematosus, cdle chronic discoid le, chron discoid lupus erythemat, lupus, lupus erythematosus discoid diagnosis, lupus erythematosus discoid, discoid lupus. Lupus erythematosus le is a chronic, autoimmune, multisystem disease that displays many diverse symptoms in which localized cutaneous le cle is on. He also used the term lupus erythematosus and published the fi rst illustrations in his. The main types of lupus are discoid lupus erythematosus dle, subacute cutaneous lupus erythematosus scle and systemic lupus erythematosus. Systemic lupus erythematosus sle is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations.
An individuals own immune system attacks various cells causing a wide variety of signs and symptoms. The clasi cutaneous lupus erythematosus disease area. Discoid lupus erythematosus, which mainly affects the skin. The clinical course is variable, with a broad spectrum of organ manifestations. Systemic lupus erythematosus sle or lupus is a chronic, progressive, autoimmune disorder that affects multiple organ systems, with a broad range of clinical and laboratory manifestations.
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