Lupus erythematosus le is a chronic, autoimmune, multisystem disease that displays many diverse symptoms in which localized cutaneous le cle is on. The rash of acute cutaneous lupus erythematosus can be transient and heal without scarring, although persistently active rashes may result in. Th e systemic form was further established by osler in baltimore and jadassohn in vienna. The american college of rheumatology acr established 11 criteria as a classificatory instrument to operationalise the definition of sle in clinical trials. Guideline on clinical investigation of medicinal products for the. Systemic lupus erythematosus, referred to as sle or lupus, is a chronic longterm disease that causes systemic inflammation which affects multiple organs. Systemic lupus erythematosus sle sle is a chronic autoimmune disease that can a. People of all races and ethnic groups can develop lupus. Chronic cutaneous lupus erythematosus ccle is more common in women, affecting from 1.
Subacute cutaneous lupus erythematosus observed by a clinician. Dubois lupus erythematosus and related syndromes ninth. 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. An increasing number of drugs have been linked to druginduced subacute cutaneous lupus erythematosus discle.
Pdf cutaneous lupus erythematosus issues in diagnosis and. Disease severity is wide ranging, with most suffering milder. Cutaneous lupus erythematosus piell latinoamericana. Sle is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs. Jun 19, 2015 systemic lupus erythematosus sle is an autoimmune disease with a prevalence of 36. Cutaneous lupus erythematosus definition of cutaneous 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.
Cutaneous lupus erythematosus cle is lupus affecting the skin. Systemic lupus erythematosus, lahita rg ed, churchill livingstone, new york 1992. The clinical course is variable, with a broad spectrum of organ manifestations. Oct 11, 2016 cutaneous lupus erythematosus 3 categories of lespecific skin diseases 1. Systemic lupus erythematosus diagnosis and treatment. A patient may be diagnosed with cutaneous lupus, but that does not mean that he or she has sle, which affects multiple parts of the body. 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. The aim of this chapter, which is set out as below, is to highlight the key features of these conditions. 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.
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. Cutaneous lupus erythematosus cle is a heterogeneous autoimmune skin disease that can present with or without systemic lupus erythematosus sle. 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. The lupus foundation of america has an information page on lupus and antiphospholipid antibodies. Systemic lupus erythematosus sle is an autoimmune disease with a prevalence of 36. In this autoimmune disease, the bodys immune system attacks healthy skin. Many people with lupus may have skin problems, and a rash may be the first sign of the condition. Cutaneous lupus erythematosus clinical and laboratory. Chronic cutaneous lu pus erythematosus comprises discoid lupus erythematosus dle and lupus profundus lp. The 2019 eularacr classification criteria for sle include positive ana at least once as. Apoptosis in chronic cutaneous lupus erythematosus, discoid lupus.
Discoid lupus erythematosus, which mainly affects the skin. Cutaneous manifestations of systemic lupus erythematosus. Cutaneous lupus refers to a form of the disease in which symptoms are restricted to those that affect the skin. He also used the term lupus erythematosus and published the fi rst illustrations in his. From basic scientific principles to practical points of clinical management, the updated 9th edition provides extensive, authoritative coverage of systemic lupus. Lupus erythematosus le comprises an uncommon group of connective tissue disorders. Cutaneous lupus erythematosus le may present in a variety of clinical forms. Click on lupus foundation of america to view the information page. Acute cutaneous lupus erythematosus acle may occur as a localized, occasionally transient, or generalized widespread form.
Sontheimer2 1 private dermatology practice, coralville, iowa, usa 2 department of dermatology, university of utah school of medicine, salt lake city, utah, usa. 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. All forms of skin rashes in lupus are made worse by exposure to light particularly sunlight. 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. Pdf cutaneous lupus erythematosus issues in diagnosis. An individuals own immune system attacks various cells causing a wide variety of signs and symptoms. Lupus information sheet continued lupus strikes mostly women of childbearing age 1544. Apr 14, 2020 subacute cutaneous lupus erythematosus scle is a nonscarring, nonatrophyproducing, photosensitive dermatosis. Lupus erythematosus le is a chronic, autoimmune disease that includes a broad spectrum of symptoms. There are over 100 medications that are known to cause lupuslike side effects in certain people.
Three recognized subtypes of cutaneous le are acute cutaneous le acle, subacute cutaneous le scle, and chronic cutaneous le ccle. 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. 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 genetic and rare diseases. American college of rheumatology classification criteria for. Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli. Lupus erythematosus le is a connective tissue disease and autoimmune disorder that can affect one or several organs. Acle may be localized most often as a malar or butterfly rash or generalized.
The rashes seen in lupus can be specific in shape and pattern. The disease has been studied in dogs as the animal model for sle in humans. Managing cle is important in order to reduce potential and established areas of damage, as well as improve quality of life qol. The autoimmune disease lupus erythematosus le is associated with a broad range of cutaneous pathology. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels. It has been considered the great imitator of many different diseases. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli cated and challenging disease to diagnose and treat. Pdf cutaneous lupus erythematosus le may present in a variety of clinical forms. Cutaneous lupus erythematosus encompasses a wide range of dermatologic. 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. The term cutaneous lupus erythematosus is applied to patients with cutaneous lesions produced by lupus erythematosus, whether involvement is exclusively cutaneous or part of a systemic disease. Subacute cutaneous lupus erythematosus scle is a nonscarring, nonatrophyproducing, photosensitive dermatosis. Circulating autoantibodies and immune complexes are due to loss of normal immune tolerance and are pathogenic. With lupus, the immune system misfires and attacks self, the patients own tissues, in a.
Cutaneous lupus erythematosus images codes and concepts open go to the image library. The underlying cause of le is unknown but the etiology is thought to be multifactorial. When healthy, our immune system protects the body from germs and cancers. 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. 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. It is a common skin manifestation in druginduced lupus.
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. With regards to the skin, there are lupus specific skin lesions and nonspecific skin lesions. Cutaneous lupus erythematosus is categorized as acute, subacute, and chronic. As with sle, subacute cutaneous lupus erythematosus scle occurs primarily in young to middle aged women. Systemic lupus erythematosus is a systemic autoimmune disease with a worldwide distribution. There is a very small chance that these cutaneous forms of lupus may develop into systemic lupus erythematosus sle with internal organ involvement. It is important to distinguish cutaneous lupus from systemic lupus erythematosus sle. However, men, children, and teenagers develop lupus, too. 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. This is a pdf file of an unedited manuscript that has. Systemic lupus erythematosus sle is a complex autoim. Although both men and women of all age groups can be affected, women outnumber men almost 10 fold and. The 10 year survival rate is exceeding 85% according to recent reports. Three recognized subtypes of cutaneous le are acute cutaneous le.
Dubois lupus erythematosus and related syndromes ninth edition. Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs. Lupus, cutaneous cle and systemic lupus erythematosus sle. Women of color are 23 times more likely to develop lupus. The symptoms of this form of lupus include a rash on the face, scalp, or elsewhere. Scle commonly develops in sunexposed areas, including the upper back, shoulders, extensor arms, neck, and upper torso, while the face is often spared. 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. Scle is highly photosensitive, with 7090% of patients meeting the acr definition of abnormal photosensitivity. Lupus strikes 1 in 400 white woman and 1 in 250 black women, it is also common in china.
The clinical management of systemic lupus erythematosus, second edition, schur ph ed, lippincott, philadelphia 1996. Symptoms vary between people and may be mild to severe. The clasi cutaneous lupus erythematosus disease area and. Lupus erythematosus primary care dermatology society uk. The diagnosis and treatment of systemic lupus erythematosus. Systemic lupus erythematosus sle is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. Subacute cutaneous lupus erythematosus is another lupusspecific rash. Dle usually only affects the skin but, in some cases, it can progress to the bodys tissues and and organs systemic lupus erythematosus see below. Clinical characteristics of each group are unique 8. Dubois lupus erythematosus and related syndromes has been recognized internationally as the goto clinical reference on lupus and other connective tissue diseases. 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 progressive autoimmune disease that results in inflammation and tissue damage characterized by flares, spontaneous remission, and relapses, sle is a chronic disease. Lupus was fi rst recognised as a systemic disease with visceral manifestations by moriz kaposi 18371902.
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. 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. Subacute cutaneous lupus erythematosus scle workup. Cutaneous lupus erythematosus issues in diagnosis and treatment hobart w. Systemic lupus erythematosus sle, is the most common type of lupus. Cutaneous lupus erythematosus 3 categories of lespecific skin diseases 1. Nondrug therapy should be used in every case, which includes photoprotection, smoking cessation, and avoiding drugs. Lupus caused by medication is known as druginduced lupus. Approximately twothirds of people with lupus will develop some type of skin disease, called cutaneous lupus erythematosus. Systemic means that it affects many organs of the body. The name systemic lupus erythematosus dates back to the early 20th century. Nondrug therapy should be used in every case, which includes photoprotection, smoking cessation, and. Subacute cutaneous lupus erythematosus scle differential.