DiscoidSCLEProfundusAssociation with systemic lupus erythemACLEAcuteCCLEDisordersEtiologyDermatomyositisInflammatoryDiagnosisAssociated with systemicConnective Tissue DUrticarial vasculitisManifestationsTumid lupusLesionsErythema nodosumPatients with relapsing polySymptomsIncidenceVasculitisHeterogeneousSubtypesSyndromeDiseasesAbnormalitiesClinicalManifestTypicallyDiseaseMutationInvolvesLesionImmunePrevalenceInvolvementSkinReactionsPathogenesisCommonlyHistopathologicalDermatologySchedel
Discoid17
- Lupus has four main types:[citation needed] systemic discoid drug-induced neonatal Of these, systemic lupus erythematosus (also known as SLE) is the most common and serious form. (wikipedia.org)
- The current version includes several subsets of cutaneous lupus erythematosus (CLE): acute (ACLE) (bullous SLE, toxic epidermal necrolysis), subacute (SCLE), chronic cutaneous lupus (CCLE) PIK3R1 (discoid lupus, lupus panniculitis, lupus erythematosus tumidus, and lupus chilblain), presence of oral or nasal ulcers, and noncicatricial alopecia [3]. (oscars2019info.com)
- However, B cells are increasingly considered to have additional functions, supported by studies finding them to occur in highest numbers in chronic discoid lupus erythematosus (CDLE), a subtype in which autoantibodies are often absent. (frontiersin.org)
- CCLE can be further subdivided into chronic discoid lupus erythematosus (CDLE), lupus erythematosus profundus (LEP) and chilblain lupus erythematosus (ChLE), of which CDLE represents the most frequent CCLE subtype ( 4 ). (frontiersin.org)
- Lupus erythematosus, discoid LE, systemic LE. (cyberderm.net)
- In discoid lupus erythematosus persistent several mm to cm sharply bordered, sometimes confluent, and painful lesions with triad of erythema, follicular keratotic plugs, and atrophy (each of which can dominate clinical picture). (cyberderm.net)
- Pure cutaneous disease usually chronic with hypopigmentation and scarring, especially when discoid. (cyberderm.net)
- Discoid lupus erythematosus (DLE) is a disfiguring autoimmune skin disease and the most common form of chronic cutaneous lupus erythematosus. (logicalimages.com)
- Discoid rash is one of the 11 diagnostic criteria for systemic lupus erythematosus (SLE), and 20% of patients with SLE will manifest discoid lesions. (logicalimages.com)
- Chronic cutaneous lupus erythematosus (CCLE) can be further divided into 3 main types: discoid lupus erythematosus (DLE), tumid lupus, and lupus panniculitis. (medscape.com)
- Discoid lupus erythematosus on the face. (medscape.com)
- Chronic scarred lesion of discoid lupus erythematosus. (medscape.com)
- However, the exact etiology of discoid lupus erythematosus (DLE) is not well understood. (medscape.com)
- Discoid lupus erythematosus (DLE) is responsible for 50-85% of cases of CLE and occurs 2-3 times more frequently in women than in men. (medscape.com)
- Although the prognosis of patients with discoid lupus erythematosus (DLE) is favorable regarding mortality, morbidity can be considerable. (medscape.com)
- Age-standardized incidence rates stratified by sex and race were calculated for discoid lupus erythematosus (DLE) in particular and for CCLE in general. (cdc.gov)
- CCLE comprises discoid lupus erythematosus (DLE), lupus erythematosus profundus (LEP), lupus erythematous tumidus (LET) and chilblain lupus erythematosus (CHLE). (cdc.gov)
SCLE3
- CLE can be classified based on clinical and histopathologic findings: typical morphological subsets are acute cutaneous (ACLE), subacute cutaneous (SCLE), intermittent cutaneous [ICLE, also termed lupus erythematosus tumidus (LET)], and chronic cutaneous (CCLE) lupus erythematosus ( 2 , 3 ). (frontiersin.org)
- This syndrome has been described in patients with DLE, subacute cutaneous lupus erythematosus (SCLE), and SLE. (logicalimages.com)
- Subacute cutaneous lupus erythematosus (SCLE) characteristically presents as annular or psoriasiform plaques in a photodistribution. (medscape.com)
Profundus1
- CCLE: Epidermal atrophy, hyperkeratosis (primarily follicular), vacuolar degeneration of basal keratinocytes, thickened basement membrane zone (PAS stain), edema, dense lymphocytic perivascular and periadnexal infiltrate at all levels, and, including subcutaneous fat in lupus profundus. (cyberderm.net)
Association with systemic lupus erythem1
- DLE may occur in the absence of systemic disease, or it may occur in association with systemic lupus erythematosus (SLE). (medscape.com)
ACLE1
- Acute cutaneous lupus erythematosus (ACLE) most commonly presents as symmetric erythema overlying the malar cheeks and nasal bridge with sparing of the nasolabial folds (butterfly rash). (medscape.com)
Acute1
- Cutaneous lupus erythematosus (CLE) can be divided into 3 main subtypes: acute, subacute, and chronic, all of which demonstrate photosensitivity. (medscape.com)
CCLE1
- Relative to SLE, epidemiologic studies on chronic cutaneous lupus erythematosus (CCLE) are rare and limited to populations without racial diversity. (cdc.gov)
Disorders5
- Our center is recognized for excellence in the diagnosis and management of a variety of inflammatory and autoimmune disorders that affect the joints, muscles, skin, blood vessels, brain, and other vital organs. (clevelandclinic.org)
- Rheumatic and autoimmune diseases are disorders which can affect any part of the body including the joints, muscles, connective tissues, and soft tissues around the joints and bones. (clevelandclinic.org)
- Medical care must include assessment for and treatment of other confounding or concurrent autoimmune disorders. (medscape.com)
- Wierzbicka et al reported good airway patency for more than 24 months in eight of 12 patients with relapsing polychondritis or other autoimmune disorders treated with this approach. (medscape.com)
- Cutaneous lupus erythematosus (CLE) comprises multiple dermatological disorders, which may be skin-limited or found in association with underlying systemic lupus erythematosus (SLE). (cdc.gov)
Etiology4
- The evidence for an autoimmune etiology includes pathological findings of infiltrating T cells, the presence of antigen-antibody complexes in affected cartilage, cellular and humoral responses against collagen type II and other collagen antigens, and the observation that immunosuppressive regimens most often suppress the disease. (medscape.com)
- CONCLUSION: The presence of ghost adipocytes is a rather unique histopathological feature consistent with pancreatic panniculitis and should be considered in combination with the overall clinical picture to determine the underlying etiology. (bvsalud.org)
- The present study aimed to report all the cases of children admitted to our tertiary pediatric hospital with the diagnosis of EN to evaluate the epidemiology, clinical manifestations, etiology, treatment, and the course of this disease in the pediatric age. (bvsalud.org)
- Systemic lupus erythematosus is a chronic, multisystem, inflammatory disorder of autoimmune etiology, occurring predominantly in young women. (merckmanuals.com)
Dermatomyositis1
- Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous findings that occur in children and adults (see the image below). (medscape.com)
Inflammatory4
- Chronic inflammatory autoimmune disease with marked immunogenetic component which involves the skin and/or other organs. (cyberderm.net)
- Inflammation in lupus is caused by the formation of autoantibodies and immune complexes and can cause inflammatory responses and activate the complement system. (bmj.com)
- type 1 autoimmune pancreatitis , interstitial nephritis , Riedel's thyroiditis , Mikulicz's disease , Küttner's tumor , inflammatory pseudotumors (in various sites of the body), mediastinal fibrosis and some cases of retroperitoneal fibrosis . (mdwiki.org)
- Many types of inflammatory dermatoses or cutaneous injuries can cause changes in pigmentation. (barefacedtruth.com)
Diagnosis7
- The Center for Pediatric Rheumatology provides comprehensive diagnosis, treatment, and follow-up care for children and adolescents who are living with juvenile arthritis and a wide range of autoimmune and rheumatologic diseases. (clevelandclinic.org)
- Diagnosis of lupus will vary from person to person. (wikipedia.org)
- Diagnosis of lupus erythematosus requires a physical examination, blood and urine tests, and a skin or kidney biopsy. (wikipedia.org)
- This observational study retrospectively considered all children evaluated to the emergency room (ER) of Meyer Children's University Hospital, Florence, Italy, discharged with a diagnosis of EN over a 12-year period (from January 2009 to December 2021). (bvsalud.org)
- The etiologic diagnosis of EN was made in 38 children (55.9%): 29 (42.6%) had infection-related EN (in particular EBV and ß-hemolytic streptococcus), 6 (8.8%) had Crohn's disease, 1 celiac disease, 1 Sjogren syndrome, and 1 Hodgkin lymphoma. (bvsalud.org)
- In 30 patients (45%), no definitive diagnosis was reached, and they were defined as having idiopathic EN. (bvsalud.org)
- The diagnosis can be difficult, particularly in the early stages, because many of the symptoms are very common and require differentiation with other diseases, especially connective tissue, such as systemic lupus erythematosus. (epainassist.com)
Associated with systemic2
- Nephrology: nephritis, nephritic proteinuria associated with systemic autoimmune disease. (clevelandclinic.org)
- Cutaneous lupus erythematosus (CLE) is an interferon (IFN)-driven autoimmune disease that may be limited to the skin or can be associated with systemic lupus erythematosus (SLE). (frontiersin.org)
Connective Tissue D1
- Treatment of calcinosis - Treatment of calcinosis cutis associated with autoimmune connective tissue diseases involves careful consideration of the need for treatment and the selection of an appropriate treatment regimen. (medilib.ir)
Urticarial vasculitis1
- Treatment of refractory hypocomplementaemic urticarial vasculitis syndrome progressing to systemic lupus erythematosus with belimumab. (uk-erlangen.de)
Manifestations7
- Patients with juvenile-onset lupus are more vulnerable to mucocutaneous manifestations of the disease (alopecia, skin rash, and ulceration of the mucus membranes) than any other age group, and they are also more susceptible to evaluation of pulmonary artery pressure. (wikipedia.org)
- Such importance is evidenced in the structure of the new SLICC (Systemic Lupus International Collaborating Clinics) criteria set for the classification of patients with SLE, Ivermectin which has expanded the catalog of skin manifestations as criteria to ease the classification of otherwise nonclassifiable SLE patients. (oscars2019info.com)
- Cutaneous manifestations of systemic disease may be the initial complaint that causes patients to seek out a healthcare provider. (medscape.com)
- Overall mortality rates have been reported to be 48% and 20% for patients with and without cutaneous manifestations, respectively. (medscape.com)
- Germline HAVCR2 mutations are frequently detected in subcutaneous panniculitis-like T-cell lymphoma (SPTCL) patients with/without hemophagocytic lymphohistiocytosis (HLH) but factors associated with variable manifestations remain undetermined. (bvsalud.org)
- Common manifestations may include arthralgias and arthritis, Raynaud syndrome, malar and other rashes, pleuritis or pericarditis, renal or central nervous system involvement, and autoimmune cytopenias. (merckmanuals.com)
- 15 The key role of aberrant B cell autoreactivity in SLE was revealed in a landmark murine study using a knockout gene mutation to prevent lupus mice from developing B cells, which resulted in a lack of autoantibody formation and clinical manifestations (nephritis or vasculitis). (bmj.com)
Tumid lupus1
- Tumid lupus typically presents with juicy papules and plaques that heal without scarring, whereas lupus panniculitis involves the subcutaneous tissue, leading to painful subcutaneous nodules that heal with depression and atrophy. (medscape.com)
Lesions4
- Lupus erythematosus (LE) skin lesions typically feature a histopathologic pattern termed interface dermatitis, defined by the presence of necroptotic keratinocytes and an epitheliotropic cytotoxic lymphocytic infiltrate at the dermo-epidermal junction ( 7 , 8 ). (frontiersin.org)
- Polyclonal B-cell activation with autoantibody formation: direct pathogenic antibodies (autoimmune haemolytic anaemia), circulating immune complexes (vasculitis, nephritis), antibody-mediated cytotoxicity (skin lesions). (cyberderm.net)
- The presence of erythema multiforme -like lesions in a patient with lupus, along with a speckled pattern of antinuclear antibody (ANA), positive anti-Ro/SSA or anti-La/SSB, and positive rheumatoid factor (RF) is known as Rowell syndrome. (logicalimages.com)
- Limited cutaneous lesions are more common than severe necrotizing complications, which are rare. (rheumaknowledgy.com)
Erythema nodosum2
- Erythema nodosum (EN), although relatively uncommon in the pediatric population, is the most frequent type of panniculitis in children. (bvsalud.org)
- Erythema Nodosum Erythema nodosum is a specific form of panniculitis characterized by tender, red or violet, palpable, subcutaneous nodules on the shins and occasionally other locations. (msdmanuals.com)
Patients with relapsing poly1
- Furthermore, levels of antibodies to matrilin 1, an extracellular matrix protein predominantly expressed in tracheal cartilage, were significantly higher in patients with relapsing polychondritis, especially in those with respiratory symptoms, than in patients with Wegener granulomatosis , systemic lupus erythematosus , or RA and in healthy controls. (medscape.com)
Symptoms2
- It is common to be diagnosed with other illnesses before a doctor can finally rule out lupus because a lot of the symptoms overlap with other common illness. (wikipedia.org)
- Therefore, for many patients, the primary goal of treatment is to minimize symptoms and alleviate functional limitations rather than the complete resolution of cutaneous calcification [ 1 ]. (medilib.ir)
Incidence1
- In a 2009 study from Olmstead County, Minnesota, Durosaro et al demonstrated that the incidence of cutaneous lupus erythematosus (CLE) is comparable to that of SLE. (medscape.com)
Vasculitis4
- that is, nodular vasculitis is a form of lobular panniculitis associated with subcutaneous blood vessel vasculitis that leads to ischemic changes. (medscape.com)
- Although erythema induratum and nodular vasculitis were once considered to be the same disease, nodular vasculitis is now considered a multifactorial syndrome of lobular panniculitis in which tuberculosis may or may not be one of a multitude of etiologic components. (medscape.com)
- Non- specific changes include erythematous maculopapular exanthema, diffuse hair loss, Raynaud syndrome, livedo racemosa (anticardiolipin antibodies and others) as a sign of cutaneous vasculitis, can all be present. (cyberderm.net)
- Effective and safe treatment of anti-CD38 therapy in systemic lupus erythematosus-associated refractory cerebral vasculitis induces immune tolerance. (uk-erlangen.de)
Heterogeneous1
- Cutaneous lupus erythematosus (CLE) is a heterogeneous autoimmune skin disease that can occur isolated to the skin or with additional systemic manifestation in several organs [systemic lupus erythematosus (SLE)] ( 1 ). (frontiersin.org)
Subtypes1
- Lupus erythematosus is a polygenic autoimmune disease linked to various HLA subtypes, immune signaling, and environmental factors, which ultimately leads to autoantibody production and T-cell dysfunction. (medscape.com)
Syndrome2
- Hematology: autoimmune cytopenia, autoimmune lymphoproliferative syndrome (ALPS). (clevelandclinic.org)
- Some drugs (eg, hydralazine , procainamide , isoniazid , anti-tumor necrosis factor [TNF] drugs) cause a reversible lupus-like syndrome. (merckmanuals.com)
Diseases2
- Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. (wikipedia.org)
- Effects of casirivimab/imdevimab on systemic and mucosal immunity against SARS-CoV-2 in B-cell depleted patients with autoimmune rheumatic diseases refractory to vaccination. (uk-erlangen.de)
Abnormalities1
- This is BFT's final installment in our series on cutaneous dyschromias - abnormalities of skin pigmentation. (barefacedtruth.com)
Clinical1
- Clinical Images: Tissue remodeling in autoimmune statin-induced necrotizing autoimmune myopathy. (uk-erlangen.de)
Manifest1
- Some other tests that may need to be run include: Antinuclear antibody (ANA) CBC with differential Chest X-ray Serum creatinine Urinalysis Lupus erythematosus may manifest as systemic disease or in a purely cutaneous form also known as incomplete lupus erythematosus. (wikipedia.org)
Typically1
- Causes of photosensitivity may include: change in autoantibody location cytotoxicity induction of apoptosis with autoantigens in apoptotic blebs upregulation of adhesion molecules and cytokines induction of nitric oxide synthase expression ultraviolet-generated antigenic DNA It is typically believed that lupus is influenced by multiple genes. (wikipedia.org)
Disease12
- Photosensitivity is a known symptom of lupus, but its relationship to and influence on other aspects of the disease remain to be defined. (wikipedia.org)
- Atherosclerotic cardiovascular disease is more prevalent in African Americans with lupus than in Caucasians with lupus. (wikipedia.org)
- Autoimmune disease of the skin and multiple internal organs. (dermacompass.net)
- Individuals of African and Hispanic descent are at increased risk, and there may be a positive family history of lupus or connective tissue disease. (logicalimages.com)
- Pamphlets from the Lupus Foundation of America, Inc. concerning skin disease and photosensitivity are useful adjuncts to verbally delivered information. (medscape.com)
- INTRODUCTION: Pancreatic panniculitis is a rare skin manifestation of pancreatic disease. (bvsalud.org)
- Pancreatic panniculitis can be a primary presenting feature and possible complication of pancreatic disease. (bvsalud.org)
- Rheumatoid Arthritis (RA) Rheumatoid arthritis is a chronic systemic autoimmune disease that primarily involves the joints. (merckmanuals.com)
- Treatment principles - The treatment of dystrophic calcinosis cutis associated with autoimmune connective tissue disease is often difficult. (medilib.ir)
- Is the underlying autoimmune connective tissue disease adequately treated? (medilib.ir)
- Treatment of underlying disease - Suppression of activity of the associated autoimmune disease is suggested based upon the theory that autoimmune disease-related tissue injury contributes to risk for cutaneous calcification ( algorithm 1 ) [ 2 ]. (medilib.ir)
- Psoriatic arthritis is an autoimmune disease that attacks the joints. (100arthritis.com)
Mutation2
- Recently, we have implemented and successfully applied a method based on genotype imputation of rare variation, on a set of genes detected by exome sequencing as possible candidates for association to systemic lupus erythematosus (SLE) by mutation in members of Icelandic SLE-multicase families ( 15 ). (frontiersin.org)
- Six cases with HAVCR2Y82C mutation manifested with HLH without panniculitis. (bvsalud.org)
Involves1
- Systemic lupus erythematosus (SLE) is a complex connective tissue disorder that involves multiple organs. (bmj.com)
Lesion1
- FDG uptake in the panniculitis lesion is rarely reported in the literature. (bvsalud.org)
Immune1
- Usually, these factors contribute to lupus by influencing the immune system. (wikipedia.org)
Prevalence1
- Worldwide, the prevalence of systemic lupus erythematosus (SLE) ranges from 17-48 cases per 100,000 population. (medscape.com)
Involvement1
- Depends on systemic involvement (CNS lupus, lupus nephritis). (cyberderm.net)
Skin2
- in SLE also in sun-exposed normal skin (lupus band test). (cyberderm.net)
- Skin excisional biopsy was performed, and the results suggested panniculitis. (bvsalud.org)
Reactions2
- SLE may be precipitated by currently unknown environmental triggers that cause autoimmune reactions in genetically predisposed people. (merckmanuals.com)
- PIH may also result from hypersensitivity reactions to medications, cutaneous injury from irritants, burns, or cosmetic procedures. (barefacedtruth.com)
Pathogenesis2
- however, the pathogenesis is autoimmune. (medscape.com)
- The specificity of autoimmune injury to cartilaginous tissues has led investigators to test the hypothesis that a cartilage-specific autoantibody is central to the pathogenesis of relapsing polychondritis. (medscape.com)
Commonly2
- Other genes that are commonly thought to be associated with lupus are those in the human leukocyte antigen (HLA) family. (wikipedia.org)
- Lupus can develop in people at any age, but it does most commonly at ages 15 to 44, with varying results. (wikipedia.org)
Histopathological1
- 1 The first report of lupus in the eye was in 1929, and Semon and Wolff, in 1933, described the histopathological characteristics of choroiditis and subretinal exudation. (bmj.com)
Dermatology1
- The Georgia Lupus Registry used multiple sources for case finding, including dermatology and rheumatology practices, multispecialty healthcare facilities, and dermatopathology reports. (cdc.gov)
Schedel1
- Lupus erythematosus was first described and distinguished from lupus vulgaris by Cazenave and Schedel in 1833. (bmj.com)