Clinical characteristics of each group are unique 8. Most common form of chronic cutaneous lupus erythematosus. Discoid le dle is the most common chronic manifestation of le. Systemic lupus erythematosus is a remarkable and challenging disorder. Le is included among the so called connective tissue diseases and is divided into one systemic form sle and one cutaneous form cle. It has a prolonged course and can have a considerable effect on quality of life. Discoid lupus erythematosus symptoms, diagnosis and. Cutaneous lupus can be part of systemic lupus or it can be a condition on its own. Discoid lupus erythematosus is a chronic dermatological disease that can lead to scarring, hair loss, and hyperpigmentation changes in skin if it is not treated early and promptly. However, there are some atypical clinical variants that may mimic other dermatological conditions. Lesions are welldemarcated erythematous macules or papules with a scaly surface, which frequently evolve into larger coinshaped plaques.
Racial disparities in the incidence of primary chronic. Th e figure 1 natural history of systemic lupus erythematosus. Eightysix patients with chronic cutaneous lupus erythematosus were examined. Cutaneous manifestations the spectrum of cutaneous manifestations of lupus erythematosus le is broad, but the most classical forms associated with systemic le are the acute malar and chronic discoid lupus erythematosus dle rash. Hence, based upon the clinical symptoms and histopathological findings she was diagnosed as a case of chronic cutaneous lupus erythematosus with systemic symptoms. Tumid lupus erythematosus tle, also known as lupus erythematosus tumidus, is a highly photosensitive form of cutaneous lupus erythematosus cutaneous. We sought to provide minimum estimates of the incidence of primary ccle ccle in the absence of sle in a population comprised predominantly of white.
Systemic lupus erythematosus sle, is the most common type of lupus. Lupus erythematosus tumidus let is a form of chronic cutaneous lupus which presents with erythematous urticaria like plaques on sunexposed sites. Clinical and laboratory findings in seventeen patients. Patients may develop residual hyperpigmentation, and scars or milia may occasionally form. A form caused by exposure to a drug and resolving with discontinuance of the drug, characterized by signs and symptoms similar to those of systemic lupus.
Chronic cutaneous lupus erythematosus ccle is a form of cutaneous lupus erythematosus cle that includes five different forms. Cutaneous lupus was the first type of lupus to be diagnosed. Coexistence of chronic cutaneous lupus erythematosus and. Her condition consisted of violaceous papules and plaques with some focal atrophy distributed over the upper arms, face and scalp. Lupus disease definition of lupus disease by medical. Perivascular and periadnexal lymphocytic infiltration and interstitial mucin deposition are the main microscopic characteristics. 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 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.
Depression may occur in up to 30% of individuals with cutaneous lupus erythematosus cle, many of whom may also have systemic manifestations. Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. Over time, only 5% of patients presenting as chronic cutaneous lupus evolve to the systemic form. Systemic lupus erythematosus is an autoimmune disease that affects many systems, including the skin, musculoskel. Cutaneous lupus erythematosus comprises a range of dermatologic manifestations, including acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. Lupus erythematosus le is a chronic, autoimmune disease that includes a broad spectrum of symptoms. Chronic cutaneous lupus erythematosus ccle conditions are less likely to present systemic involvement than acute and subacute conditions but more often cause permanent scarring and dyspigmentation. Lupus also occurs in a form limited to the skin, usually as discoid lupus. The diagnosis of sle was not made as although the clinical criteria were fulfilled but no immunological criteria was present.
We found that 26% of individuals with chronic cutaneous lupus erythematosus ccle reported moderate to severe depressive symptoms. Chronic cutaneous lupus erythematosus, or simply discoid lupus, is an indolent skin disease usually occurring between age 20 and 45 years. Chronic cutaneous lupus erythematosus ccle most commonly presents in the form of discoid lupus erythematosus dle with pinktoviolaceous coinshaped plaques with hyperkeratosis and follicular plugging that heal with atrophy, scarring, hyperpigmentation, and. The most common forms are subacute cutaneous lupus erythematosus scle and discoid lupus erythematosus dle. Lupus is the latin word for wolf and has been used to name various skin diseases at least since the 10 th century. Request pdf chronic cutaneous lupus erythematosus skin, to a similar extent as the joints, represents the most common organ involved in le either as primary manifestation or during the course. Comparative analysis of subacute cutaneous lupus erythematosus and chronic cutaneous lupus erythematosus. Chronic cutaneous lupus erythematosus presenting as. The typical clinical manifestation of chronic cutaneous lupus erythematosus ccle is the chronic discoid lupus erythematosus.
Relative to studies of systemic lupus erythematosus sle, epidemiologic studies of chronic cutaneous lupus erythematosus ccle are rare and are limited to populations with no racial diversity. The acr criteria, which include four cutaneous signs, may lead to overdiagnosis of sle in patients with predominantly cutaneous disease. Chronic cutaneous lupus erythematosus springerlink. Chronic cutaneous lupus erythematosus ccle usually presents as characteristic erythematous patches and infiltrated coinshaped plaques. We strongly recommend that you talk with a trusted. Lupus tumidus is considered a rare subtype of chronic cutaneous lupus erythematosus, characterized by erythema and bright urticarial erythematous violaceous. Background skin disorders can cause mental health problems associated with stigmatization and social isolation. Sle is an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs.
Chronic cutaneous lupus erythematosus ccle is an autoimmune disease that is associated with autoantibody production and tcell dysfunction. Discoid lupus erythematosus is the most common form of cutaneous lupus erythematosus. Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs. The sequelae of chronic cutaneous lupus erythematosus. Cutaneous sarcoidosis masquerading as chronic cutaneous.
This type affects the skin and can cause thick, red, scaly rashes on the face, neck, and scalp that can lead to scarring. Systemic lupus erythematosus in patients with chronic cutaneous discoid lupus erythematosus. Chronic cutaneous lupus erythematosus ccle is an autoimmune disease that is associated with autoantibody production and. Although there is evidence of beneficial effects of topical and systemic glucocorticoids, retinoid and chloroquine 1, there is a subgroup of patients for whom adequate disease control cannot be achieved and therefore there is an ongoing need for new treatment options. Lupus erythematosus le is an autoimmune disease characterized by a broad range of cutaneous manifestations. A 64yearold caucasian woman was seen in our office for several years for management of her chronic cutaneous lupus erythematosus ccle. Although dle usually responds to topical or intralesional corticosteroids andor oral antimalarials, some dle is resistant to. Cutaneous lupus erythematosus also known as chronic cutaneous lupus. Burge sm, frith pa, juniper rp, wojnarowska f 1989 mucosal involvement in systemic and chronic cutaneous lupus erythematosus. Chronic cutaneous lupus erythematosus is a polymorphous autoimmune disease which may mimic some other clinical conditions, causing diagnostic difficulties. Cutaneous lupus erythematosus genetic and rare diseases.
Treatment of alopecia in chronic cutaneous lupus erythematosus. Ustekinumab in the treatment of refractory chronic. The autoimmune disease lupus erythematosus le is associated with a broad range of cutaneous pathology. Although the term is frequently used alone to designate lupus vulgaris and sometimes lupus erythematosus, without a modifier it has no meaning. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels. This form is characterized by inflammatory, scarring lesions mainly involving the head or neck, but also elsewhere, mostly on the photoexposed areas. Its diversity of clinical features is matched by the complexity of the factors genetic, hormonal, and environmental that cause it, and the array of autoantibodies. There is a very small chance that these cutaneous forms of lupus may develop into systemic lupus erythematosus sle with internal organ. Cle limited cutaneous involvement to devastating systemic disease. The most common and most severe form is systemic lupus erythematosus.
Cutaneous manifestations of sarcoidosis may mimic ccle and vice versa making it difficult to reach a diagnosis clinically. Discoid lupus erythematosus dle is a common manifestation of chronic cutaneous lupus erythematosus ccle. Lupus erythematosus an overview sciencedirect topics. A chronic cutaneous form characterized by patchy lesions that form especially on the head and neck, with accompanying scarring and pigmentation changes. Lesions are often localized to the scalp and can result in permanent scarring, disfiguration, and irreversible alopecia. Importance discoid lupus erythematosus dle is a chronic variant of cutaneous lupus erythematosus, an autoimmune inflammatory disorder of the skin. Sarcoidosis is a multisystemic granulomatous disease of unknown origin. Case report chronic cutaneous lupus erythematosus with.
Often, the rashes associated with lupus occur on areas of the skin exposed to sunlight or. Coexistence of chronic cutaneous lupus erythematosus and frontal fibrosing alopecia 275 figure 3. The typical lesions in chronic discoid lupus erythematosus appear as. Cutaneous lupus erythematosus le may present in a variety of clinical forms. Early diagnosis and treatment of discoid lupus erythematosus. Cutaneous lupus erythematosus cle is lupus affecting the skin in this autoimmune disease, the bodys immune system attacks healthy skin. Phillip frost department of dermatology and cutaneous surgery, university of miami miller school of medicine, miami, fl, usa search for more papers by this author first published.
Twelve of these also suffered from systemic lupus erythematosus. C utaneous lupus erythematosus lupus in the skin includes discoid lupus and several other types of skin lupus. Cutaneous lupus erythematosus cle is a form of lupus that predominantly affects the skin. The diagnosis is usually made by clinical examination.
Slicc, systemic lupus international collaborating clinicsamerican college of rheumatology damage index. Cutaneous lupus erythematosus definition of cutaneous. Cutaneous lupus erythematosus 3 categories of lespecific skin diseases 1. Histologically, lack of epidermal alterations differentiates the let from other cutaneous lupus variants. Sle is a chronic disease of variable severity with a waxing and waning course, with signifi cant morbidity that can be fatalif not treated earlyin some patients fi gure 1. 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. Chronic cutaneous lupus erythematosus discoid lupus.
Initial clues to the subgroup, which may progress to sle, include discoid lesions above and below the neck, or the presence of polyarthralgias. Early recognition and treatment improves the prognosis. Three recognized subtypes of cutaneous le are acute cutaneous le acle, subacute cutaneous le scle, and chronic cutaneous le ccle. It is a chronic, indolent skin disease with a low incidence of systemic involvement. Chronic cutaneous lupus erythematosusclinical spectrum.
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