Lupus genetic and rare diseases information center gard. Systemic lupus erythematosus overview definition an autoimmune disease characterized by. Chronic cutaneous le accounts for 80% of presentations with cutaneous le. Subacute cutaneous lupus and acute cutaneous lupus erythematosus are highly photosensitive and are characteristically nonscarring. Cutaneous lupus erythematosus cle is a heterogeneous autoimmune skin disease that can present with or without systemic lupus erythematosus sle.
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. Concept paper for treatment of systemic and cutaneous lupus. There is no known cure, but treatments are available to help manage the symptoms. Nondrug therapy should be used in every case, which includes. A variety of skin rashes or lesions can occur with this disease, and they can range from mild to lifethreatening. The four different types of lupus lupus foundation of. Cutaneous lupus erythematosus encompasses a spectrum of cutaneous disease with different phenotypes, and it potentially intersects with many disciplines in medicine.
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 is the latin word for wolf and has been used to name various skin diseases at least since the 10 th century. Cutaneous lupus, also known as cutaneous lupus erythematosus cle, is a skin related form of the chronic autoimmune disease commonly referred to as lupus. Subacute cutaneous lupus erythematosus an overview. Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel etal, renal, neuropsychiatric, hematologic, cardiovascular, pulmonary.
Pdf cutaneous lupus erythematosus issues in diagnosis and. To date, no therapeutic agents have been licensed speci. Cle limited cutaneous involvement to devastating systemic disease. College of pharmacy, the university of texas at austin, austin, tx 78712. Systemic lupus erythematosus sle is the prototypic multisystem autoimmune disorder with a broad spectrum of clinical presentations. Cutaneous lupus erythematosus genetic and rare diseases.
It was initially reported as a hereditary disease in the german shorthaired pointer and was called lupoid dermatosis. Cutaneous lupus erythematosus definition of cutaneous lupus. Systemic lupus erythematosus sle is the prototypical systemic autoimmune disease and is characterized by a clinical course of flares and remissions. There are a number of other kinds of lupus erythematosus including discoid lupus erythematosus, neonatal lupus, and subacute cutaneous lupus erythematosus. Cutaneous lupus erythematosus encompasses a wide range of dermatologic. From basic scientific principles to practical points of clinical management, the updated 9th edition provides extensive, authoritative coverage of systemic lupus. Lupus classical discoid rashlocalised above the neck or generalised above and below the lupus panniculitis profundus.
Lupus erythematosus primary care dermatology society uk. When healthy, our immune system protects the body from germs and cancers. The lespecific cutaneous findings encompass the various subtypes of cutaneous lupus erythematosus cle and were subdivided into three different categories as defined by constellations of clinical features and average duration of the cutaneous lesions, histological changes of skin biopsy specimens, and laboratory abnormalities. Mar 22, 2016 foot complaints are common in inflammatory arthropathies such as rheumatoid arthritis and cause considerable disability. Malar eruption or butterfly rash erythema and oedema of cheeks, sparing nasolabial folds lasting hours to days. Cutaneous lupus erythematosus cutaneous le includes three subsets of lespecific skin diseases. Le is included among the so called connective tissue diseases and is divided into one systemic form sle and one cutaneous form cle. Managing cle is important in order to reduce potential and established areas of damage, as well as improve quality of life qol. About 25% of patients with chronic cutaneous le also have systemic le discoid le. The hallmark condition of chronic cutaneous lupus is discoid lupus erythematosus dle. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen lymph nodes, feeling tired, and a red rash which is. These two forms can occur concurrently in the same patient. Molecular profiling of cutaneous lupus lesions identifies. Cutaneous lupus erythematosus cle is lupus affecting the skin.
The clinical management of systemic lupus erythematosus, second edition, schur ph ed, lippincott, philadelphia 1996. The autoimmune disease lupus erythematosus is associated with a broad range of cutaneous pathology. The clinical importance of this edematous, nonscarring, photosensitive type of ccle as a distinct subset has been neglected in the literature and, since it has not always been considered as a separate entity, we suggest criteria to. 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.
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. Slicc criteria for sle slicc classification criteria for. Aug 21, 2012 cutaneous lupus erythematosus cle includes a variety of lupus erythematosus lespecific skin lesions that are subdivided into three categories chronic cle ccle, subacute cle scle and acute cle acle based on clinical morphology, average duration of skin lesions and routine histopathologic examination. Patterns of foot complaints in systemic lupus erythematosus. To develop new classification criteria for systemic lupus erythematosus sle jointly supported by the. It is more prevalent in patients with skin of colour, who are at greater risk of postinflammatory hyperpigmentation and hypertrophic scarring. 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.
Cutaneous lupus erythematosus cle is a form of lupus that predominantly affects the skin. Subacute cutaneous lupus erythematosus a twodecades perspective. However, little is published about the nature and extent of foot complaints in systemic lupus erythematosus sle. Systemic lupus erythematosus sle, also known simply as lupus, is an autoimmune disease in which the bodys immune system mistakenly attacks healthy tissue in many parts of the body.
There is a very small chance that these cutaneous forms of lupus may develop into systemic lupus. It is characterized by a discshaped or coinshaped lesion. Approximately 50% of patients with scle have underlying systemic lupus erythematosus, and about 10% of sle patients will have scle lesions in the skin. Systemic inflammatory response in many organ systems dysregulated autoimmune response involving many arms of the immune system including t cells, b cells and macrophages autoantibodies in sle. There is a very small chance that these cutaneous forms of lupus may develop into systemic lupus erythematosus sle with internal organ involvement. Average 12 normalization of cel files were performed in r. Pdf cutaneous lupus erythematosus cle includes a variety of lupus erythematosus lespecific skin lesions that are subdivided into three. Hochberg, md, mph professor of medicine, epidemiology and preventive medicine university of maryland school of medicine, baltimore, md. Chronic cutaneous lu pus erythematosus comprises discoid lupus erythematosus dle and lupus profundus lp. If you have problems viewing pdf files, download the. Subacute cutaneous lupus erythematosus may be idiopathic. A selection from the lupus foundation of america newsletter article library 93102 revised 603 the history of lupus erythematosus le has been. Systemic lupus erythematosus lupus is a chronic longlasting autoimmune disease that can affect many parts of the body.
Epub apr lupus erythematosus tumidusa neglected subset of cutaneous lupus erythematosus. Lupus erythematosus nonspecific skin lesions include nonscarring alopecia, mouth ulcers, photosensitivity, raynauds phenomenon, and vasculitisvasculopathy, among others. Subacute cutaneous lupus erythematosus scle as a distinct subset of lupus erythematosus le was first suggested by gilliam in 1977. Cutaneous lupus erythematosus, dermatological approaches. Cutaneous symptoms are often the presenting indication of lupus erythematosus le, and in the case of particular cutaneous lupus erythematosus cle subtypes, they can happen in the lack of systemic disease.
Authoritative facts about the skin from dermnet new zealand. May 02, 2016 cutaneous lupus erythematosus cle can be divided into acute cutaneous lupus, subacute cutaneous lupus, and chronic cutaneous lupus. Cutaneous lupus erythematosus clinical and laboratory. Systemic lupus erythematosus, lahita rg ed, churchill livingstone, new york 1992. Lupus erythematosus tumidus genetic and rare diseases. The autoimmune disease lupus erythematosus le is associated with a broad range of cutaneous pathology. Dubois lupus erythematosus and related syndromes ninth edition. Lupus erythematosus le is a chronic, autoimmune, multisystem disease that displays many diverse symptoms in which localized cutaneous le cle is on.
Lupus tumidus is considered a rare subtype of chronic cutaneous lupus erythematosus, characterized by erythema and bright urticarial erythematous violaceous. Although the term is frequently used alone to designate lupus vulgaris and sometimes lupus erythematosus, without a modifier it has no meaning. Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. Omeprazole and subacute cutaneous lupus erythematosus introduction omeprazole is a selective and irreversible proton pump inhibitor ppi. With lupus, the immune system misfires and attacks self, the patients own tissues, in a. In contrast to those diagnosed with sle, patients with scle tend to experience fewer occurrences of cytopenia decreased numbers of blood cells, serositis inflammation of tissues lining the. Treatment currently includes antimalarials, glucocorticoids, immunosuppressants, and biologics. In this autoimmune disease, the bodys immune system attacks healthy skin. Lupus erythematosus is usually divided into two main types. Lupus is an autoimmune disease, which affects multiple organs and systems in the body. The revised cutaneous lupus erythematosus disease area and severity index rclasi can be used to assess disease activity and damage.
Oct 11, 2016 cle limited cutaneous involvement to devastating systemic disease. Tumid erythematosus lupus is characterized by smooth, nonscarring, pink to violetcolored pimples papules on the skin without any other apparent skin changes, such as scarring. Systemic lupus erythematosus, referred to as sle or lupus, is a chronic longterm disease that causes systemic inflammation which affects multiple organs. This paper describes our personal experience in the management of cle over. Cutaneous lupus erythematosus pictures, treatment, symptoms. 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. Cutaneous lupus erythematosus cle is a disfiguring disease that can exist as an.
Diagnosis of these diseases requires proper classification of the subtype, through a combination of physical exam, laboratory studies, histology, antibody serology, and occasionally direct immunofluorescence, while ensuring to exclude systemic. Lupus is an autoimmune disease that can affect almost every organ in the body. Symptoms may include pain or swelling in joints, muscle pain, fever, red rashes, most often on the face also called the butterfly. Department of dermatology, university of texas southwestern medical center, dallas, tx 75390 background. Apoptosis in chronic cutaneous lupus erythematosus, discoid lupus. It is more common in african americans and people of american indian and asian descent than in. Lupus erythematosus tumidus, chillblains lupus, discoid lupus lichen planus overlap. Systemic lupus erythematosus, its treatment, and future. Bullous systemic lupus erythematosus successfully treated.
Lupus erythematosus is a connective tissue autoimmune disorder that demonstrates systemic, cutaneous, or both systemic and cutaneous manifestations. Lupus erythematosus le is a chronic, autoimmune disease that includes a broad spectrum of symptoms. The classification and diagnosis of cutaneous lupus erythematosus. American college of rheumatology classification criteria for. Lupus erythematosus tumidus let is a photosensitive skin disease characterized by succulent, edematous, and nonscarring plaques. Symptoms vary between people and may be mild to severe.
Lupus erythematosus le comprises an uncommon group of connective tissue disorders. Although there are many types of rashes and lesions sores caused by cutaneous lupus, the most common rash is raised, scaly and red, but not itchy. Bullous systemic lupus erythematosus bsle is a rare complication of. With regards to the skin, there are lupus specific skin lesions and nonspecific skin lesions. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for cutaneous lupus erythematosus. Tumid lupus erythematosus tle, also known as lupus erythematosus tumidus, is a highly photosensitive form of cutaneous lupus erythematosus cutaneous. Lastly, chronic cutaneous lupus can be further subdivided into a number of cutaneous findings including discoid, tumidus, profundus and chilblains. Omeprazole and subacute cutaneous lupus erythematosus. He also used the term lupus erythematosus and published the fi rst illustrations in his atlas of skin diseases in 1856. The most common forms are subacute cutaneous lupus erythematosus scle and discoid lupus erythematosus dle. Specific features of acute cutaneous le may include. We aimed to explore foot complaints among people with sle and to evaluate the associations between foot pain and selfreported activities of daily living and. Clinical characteristics of each group are unique 8.
Systemic lupus erythematosus sle is an autoimmune disease presenting with varying degrees of organ and system involvement. Cutaneous lupus is divided into several subtypes, including acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. Quality of life in patients with cutaneous lupus erythematosus cle motolani ogunsanya, b. It is commonly known as a discoid rash, because the areas of rash are. Epidemiology of systemic lupus erythematosus and cutaneous. Pdf lupus erythematosus le is a chronic, autoimmune, multisystem disease that displays many diverse symptoms in which localized cutaneous le cle.
Cutaneous lupus erythematosus is composed of four main types where each has their own distinct characteristics. The aim of this chapter, which is set out as below, is to highlight the key features of these conditions. This type of lupus can occur on any part of the body, but. Manual, pn m01226 v4 from approximately 30 ng total rna and 2. Exfoliative cutaneous lupus erythematosus basic information description exfoliative lupus erythematosus is a generalized widespread skin disease that produces a lot of dandruff or flakiness. Cutaneous lupus erythematosus cle is lupus affecting the skin in this autoimmune disease, the bodys immune system attacks healthy skin. Acute cutaneous le affects at least 50% of patients with systemic lupus erythematosus sle. Subacute cutaneous lupus erythematosus scle is characterized by the presence of nonscarring, photosensitive lesions that can take one of two distinct forms. The clasi cutaneous lupus erythematosus disease area and. Symptoms of lupus can range from very mild to lifethreatening. Cutaneous lupus skin lupus is lupus that affects the skin in the form of a rash or lesions. Dubois lupus erythematosus and related syndromes ninth. Although cutaneous lupus can cause many types of rashes and lesions sores, the most commoncalled discoid rashis raised, scaly and red, but not itchy.
The clasi cutaneous lupus erythematosus disease area. Cutaneous lupus erythematosus is categorized as acute, subacute, and chronic. Cutaneous lupus erythematosus encompasses a wide range of dermatologic manifestations, which may or may not be associated with the development of systemic disease. Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs. The four main types of cutaneous lupus erythematosus include the following. Discoid le is the most common form of chronic cutaneous le. This is a pdf file of an unedited manuscript that has. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli cated and challenging disease to diagnose and treat.
Lupus was fi rst recognised as a systemic disease with visceral manifestations by moriz kaposi 18371902. Chronic cutaneous lupus erythematosus ccle, also known as lupus erythematosus discoid, is an inflammatory disease of the skin predominantly among adults. The autoimmune disease lupus erythematosus is connected with a broad variety of cutaneous pathology. An individuals own immune system attacks various cells causing a wide variety of signs and symptoms. The lesions are classically distributed on the upper back, chest, dorsal arms, and lateral neck but also can be found in other locations. Scle subacute cutaneous lupus erythematosus uti urinary tract infection. Pdf cutaneous lupus erythematosus issues in diagnosis. Pathogenesis 20 and clinical features 1 introduction systemic lupus erythematosus sle is the prototypic multisystem autoimmune disorder with a broad spectrum of clinical presentations encompassing almost all organs and tissues. Cutaneous lupus erythematosus american skin association. Systemic lupus erythematosus sle is a heterogeneous, multisystem disease with many clinical phenotypes, including drug. Multiphase development of sle classification criteria phase 1b delphi exercise phase 1c early cohort study.
Cutaneous lupus erythematosus 3 categories of lespecific skin diseases 1. Treatments may include nsaids, corticosteroids, immunosuppressants, hydroxychloroquine, and methotrexate. The most common and most severe form is systemic lupus erythematosus. The diagnosis of systemic lupus erythematosus requires the presence of four or more of the. Click on lupus foundation of america to view the information page. Dubois lupus erythematosus and related syndromes has been recognized internationally as the goto clinical reference on lupus and other connective tissue diseases. Chronic cutaneous lupus or discoid lupus is the most common form of cutaneous lupus erythematosus. Cutaneous manifestations are frequently the presenting sign of lupus erythematosus le, and in the case of certain cutaneous lupus erythematosus cle subtypes, they can occur in the absence of systemic disease. The main types of lupus are discoid lupus erythematosus dle, subacute cutaneous lupus erythematosus scle and systemic lupus erythematosus. Cutaneous refers to the skin, and this form of lupus is limited to the skin. Chronic cutaneous lupus erythematosus includes discoid lupus erythematosus, lupus erythematosus profundus, chilblain cutaneous lupus, and lupus tumidus. Th e systemic form was further established by osler in baltimore and jadassohn in vienna. Diagnostic features on biopsy are more likely to be found in lespecific skin lesions than in lenonspecific cutaneous le. Chronic cutaneous lupus erythematosus is usually characterized by skin disease alone.
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