Symptoms vary between people and may be mild to severe. Pdf cutaneous lupus erythematosus issues in diagnosis and. Authoritative facts about the skin from dermnet new zealand. 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.
Subacute cutaneous lupus erythematosus research study. There is a very small chance that these cutaneous forms of lupus may develop into systemic lupus. Systemic lupus erythematosus sle is a multiorgan autoimmune disease of unknown etiology with many clinical manifestations. Cutaneous lupus erythematosus piell latinoamericana. The most common and most severe form is systemic lupus erythematosus. Sontheimer2 1 private dermatology practice, coralville, iowa, usa 2 department of dermatology, university of utah school of medicine, salt lake city, utah, usa. Involvement of particular organ systems over others, multisystem versus organdominant disease. The diagnosis of systemic lupus erythematosus requires the presence of four or more of the. The hallmark feature of acle is localized to the malar region butterfly rash and is characterized by confluent, macular or papular erythema lasting days to weeks that occurs symmetrically on the cheeks and bridge of the nose, sparing the nasolabial folds figure 801. 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. Three recognized subtypes of cutaneous le are acute cutaneous le acle, subacute cutaneous le scle, and chronic cutaneous le ccle. Lupus erythematosus le is a chronic, autoimmune disease that includes a broad spectrum of symptoms. Cutaneous lupus erythematosus genetic and rare diseases. Katarzyna gilekseibert, md abstract systemic lupus erythematosus sle is a chronic, compli.
Three recognized subtypes of cutaneous le are acute cutaneous le acle, subacute cutaneous le scle, and chronic. The skin is involved in up to 85% of systemic lupus erythematosus sle cases and may be the only organ involved in cutaneous lupus erythematosus cle. There is little clinical utility in combining scores because of the potential for. This is a 10 month long study involving an investigational medication for subacute cutaneous lupus erythematosus scle. Lupus is the latin word for wolf and has been used to name various skin diseases at least since the 10 th century. Druginduced lupus is a form of lupus caused by medications. Multisystem involvement as a component of systemic le sle is common, with. Clinical manifestations of cutaneous lupus erythematosus. Lupus erythematosus primary care dermatology society uk. Chronic cutaneous lupus erythematosus ccle is more common in women, affecting from 1. Cutaneous lupus erythematosus le may present in a variety of clinical forms. In numerous investigations, it has been shown that exogenous factors, such as uv.
The main types of lupus are discoid lupus erythematosus dle, subacute cutaneous lupus erythematosus scle and systemic lupus erythematosus. Lupus erythematosus is a connective tissue disease that is immune system mediated. Pdf cutaneous lupus erythematosus cle includes a variety of lupus erythematosus. Acute cutaneous lupus erythematosus acle lesions can be localized or generalized. Cutaneous lupus erythematosus cle is a potentially disfiguring autoimmune disease that can manifest only in the skin or can be present as part of a diverse range of potentially lifethreatening and debilitating symptoms in patients with lupus erythematosus le. The cutaneous lupus erythematosus disease area and severity index clasi is used as a clinical tool that standardizes the way disease activity is described and provides guidelines for identifying a clinical change. Lupus erythematosus le comprises an uncommon group of connective tissue disorders. 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. 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.
This article provides an overview of cutaneous lupus erythematosus cle. Cutaneous lupus erythematosus clinical and laboratory. The most common forms are subacute cutaneous lupus erythematosus scle and discoid lupus erythematosus dle. Subacute cutaneous lupus erythematosus scle as a distinct subset of lupus erythematosus le was first suggested by gilliam in 1977. 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 a twodecades. Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. Cutaneous lupus is divided into several subtypes, including acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus. Subacute cutaneous lupus erythematosus scle clinical. Systemic lupus erythematosus sle is a multiorgan autoimmune disease of unknown etiology with many. The diagnosis of the cutaneous manifestations of le. Chronic cutaneous le accounts for 80% of presentations with cutaneous le. As a result of clinical observations made during his early studies designed to test this hypothesis, he intro duced the term subacute cutaneous lupus erythematosus scle. Cutaneous lupus erythematosus synonyms, cutaneous lupus erythematosus pronunciation, cutaneous lupus erythematosus translation, english dictionary definition of cutaneous lupus erythematosus.
The acute cutaneous is a less severe type of lupus and manifests only on the skin. The skin is one of the target organs most variably affected by the disease. Common manifestations may include arthralgias and arthritis, raynaud syndrome, malar and other rashes, pleuritis or pericarditis, renal or central nervous system involvement, and hematologic cytopenias. Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. 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. This clinical tool quantifies disease activity and damage in cutaneous lupus erythematosus.
Click on lupus foundation of america to view the information page. Recent data have provided enhanced understanding of these interactions and the mechanism by which they cause disease. Clinical characteristics of each group are unique 8. Cutaneous lupus erythematosus issues in diagnosis and treatment hobart w. Subacute cutaneous lupus erythematosus refers to skin lesions that appear on parts of the body exposed to sun. Pdf cutaneous lupus erythematosus issues in diagnosis. As the disease evolves these lesions commonly become infiltrated and merge to make patches covered by thick scales and keratosis extending to the interior of. Lupus erythematosus is an autoimmune system disease that may take three forms. Pdf cutaneous lupus erythematosus le may present in a variety of. Cutaneous manifestations of systemic lupus erythematosus hindawi. Cutaneous lupus erythematosus 3 categories of lespecific skin diseases 1. Psoriasiform lesions with superficial scale and the tendency for individual lesions to merge.
A number of candidate genes have been identified which increase the risk of developing cle. If you have problems viewing pdf files, download the latest version of adobe reader. Ccle encompasses discoid lupus erythematosus dle, lupus erythematosus tumidus le tumidus, lupus profundus also known as. 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. Clinical manifestations of cutaneous lupus erythematosus annegret kuhn1, michael sticherling2. Cutaneous lupus erythematosus cle is a form of lupus that predominantly affects the skin. Cutaneous lupus erythematosus definition of cutaneous. Cutaneous lupus erythematosus article about cutaneous. Cutaneous lupus erythematosus cle is a chronic inflammatory autoimmune disease with a broad spectrum of clinical manifestations and a variable course. Cutaneous lupus erythematosus lupus is an autoimmune disease, which affects multiple organs and systems in the body.
Systemic lupus erythematosus sle is an autoimmune disorder affecting not just one but many organs and systems in the body like the heart, joints, skin, lungs, blood vessels, liver, kidneys and the nervous system. Study participants must be between 18 and 70 years of age and have scle. Pdf cutaneous manifestations of systemic lupus erythematosus. Systemic lupus erythematosus sle is a multiorgan autoimmune disease of unknown etiology that can have many clinical manifestations table 1. The subacute cutaneous lupus typically manifests solely on the skin, but in some cases, it may also.
A typical laboratory finding of lupus erythematosus is the presence of special cells the socalled le cells and antinuclear antibodies in the blood. The butterfly rash is specific for this condition and it will typically disappear if the patient avoids sun exposure. How to say cutaneous lupus erythematosus in english. Pronunciation of cutaneous lupus erythematosus with 1 audio pronunciation, 10 translations and more for. It is more prevalent in patients with skin of colour, who are at greater risk of postinflammatory hyperpigmentation and hypertrophic scarring. Susceptibility to develop systemic lupus erythematosus sle. Cutaneous lupus erythematosus cutaneous le includes three subsets of lespecific skin diseases. The clasi cutaneous lupus erythematosus disease area. Participants are asked to visit the study site approximately 18 times. About 25% of patients with chronic cutaneous le also have systemic le discoid le. Discoid le is the most common form of chronic cutaneous le. The pathophysiology of cutaneous lupus erythematosus cle encompasses the complex interactions between genetics, the environment, and cells and their products. Cutaneous lupus erythematosus cle is a chronic inflammatory autoimmune. Le is included among the so called connective tissue diseases and is divided into one systemic form sle and one cutaneous form cle.
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. An individuals own immune system attacks various cells causing a wide variety of signs and symptoms. Papulosquamous subacute cutaneous lupus erythematosus scle. Any of several other autoimmune disorders typically characterized by reddish, scaly lesions on the skin. Although the term is frequently used alone to designate lupus vulgaris and sometimes lupus erythematosus, without a modifier it has no meaning. Learn vocabulary, terms, and more with flashcards, games, and other study tools. Acle may be localized most often as a malar or butterfly rash or generalized. Subacute cutaneous lupus erythematosus scle is a nonscarring, nonatrophyproducing, photosensitive dermatosis. Systemic lupus erythematosus sle musculoskeletal and.
The aim of this chapter, which is set out as below, is to highlight the key features of these conditions. Cle limited cutaneous involvement to devastating systemic disease. Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs. 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. Lupus erythematosustwo types cutaneous discoid and systemic diffusecutaneous is limited to the skin.
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