Lupus erytheLupus erythematosusmatosus

DefinitionDefinition

Lupus erythematosusLupus erythematosus LE LE is classified as connective tissue disis classified as connective tissue dis

ease. It occurs predominantly in females in ease. It occurs predominantly in females in the reproductive years. Multiple abnormal ithe reproductive years. Multiple abnormal immune responses are present. mmune responses are present.

It may involved any organ of the body aIt may involved any organ of the body and is manifested in many forms. It may be lind is manifested in many forms. It may be life-threatening when it becomes severe.fe-threatening when it becomes severe.

EtiologyEtiology genetic factorsgenetic factorsthe findings of 100 LE family surveys :the findings of 100 LE family surveys : Family history positive : 12 %Family history positive : 12 % The prevalence of SLE in first-degree relatives is The prevalence of SLE in first-degree relatives is 1.84 % 1.84 % The degree of heritability was 56 %The degree of heritability was 56 % In addition, the concordance rate in monozygotiIn addition, the concordance rate in monozygotic twins comes to over 50% c twins comes to over 50% suggestionsuggestion : : LE is multifactorial inheritanceLE is multifactorial inheritance Environmental influence highEnvironmental influence high

EtiologyEtiologyEnviornmental influenceEnviornmental influence Virus infection-- other bacterial infections arVirus infection-- other bacterial infections are seldome seldom Physical factor -- sunlight ,coolness, wetnessPhysical factor -- sunlight ,coolness, wetness, ray , ray Emotional factor ---stress, worryEmotional factor ---stress, worry Endocrine factor –high estrogen ,prolactin Endocrine factor –high estrogen ,prolactin drugs -- more than 20 drugs such as penicillidrugs -- more than 20 drugs such as penicillin, phenytoin may induce symptomatic LEn, phenytoin may induce symptomatic LE

PathogenesisPathogenesisGenetic factor

Immuno-modulatory function disorder

Various auto antibody

Blood cell injured Immune-complexes precipitation

Environmental influence (infection, sunlight, drug)

Auto-antigen formed

Organs injured

Alterations in organs

Sensitivitied T- lymphocytes

Clinical manifestationClinical manifestation Discoid LE ←→Discoid LE ←→★★ Systemic LE Systemic LE

Discoid lupus erythematosusDiscoid lupus erythematosus,, 　 　 DLEDLE Skin lesions are predominantly manifestedSkin lesions are predominantly manifested Eruptions may occur singly or be multipleEruptions may occur singly or be multiple localized DLElocalized DLE Lesions localized on face, dorsa of the Lesions localized on face, dorsa of the

handhand generalized DLEgeneralized DLE Lesions widespread on trunk ,extremities Lesions widespread on trunk ,extremities

Skin lesions in discoid Skin lesions in discoid LELE

Red macules or papules associated with edeRed macules or papules associated with edemama→→ →→ discoid eyrthematosus with adherediscoid eyrthematosus with adherent scales, horny plugs—-centrally atrophy, nt scales, horny plugs—-centrally atrophy, dyspigmentation, telangiectasia dyspigmentation, telangiectasia

录象片断

skin lesions in discoid LEskin lesions in discoid LE Favorite sites are malar areas, bridge of nose, lFavorite sites are malar areas, bridge of nose, l

ower lip, ear, scalp and dorsa of the hand ower lip, ear, scalp and dorsa of the hand

录象片断

Skin lesions in discoidSkin lesions in discoid LELEatrophy in the scalpatrophy in the scalp →→false alopecia areata false alopecia areata

lesions on low lip may be erodedlesions on low lip may be eroded

Systemic lupus erythematosSystemic lupus erythematosusus SLE may involved any organ of thSLE may involved any organ of the body and is manifested in many e body and is manifested in many forms. It will be life-threatening forms. It will be life-threatening when severely.when severely. Basic pathology alteration shows Basic pathology alteration shows necrotizing vasculitisnecrotizing vasculitis

Skin and mucous Skin and mucous membrane lesionsmembrane lesions

Skin involvement occurs in 80% of cases.Skin involvement occurs in 80% of cases. It is often the earliest abnormality and predomiIt is often the earliest abnormality and predominant symptom.nant symptom. Sunlight may develop the facial eruption and a Sunlight may develop the facial eruption and a severe relapse.severe relapse. Mulity form : erythema, papule, blood blisterMulity form : erythema, papule, blood blister Specific eruptionSpecific eruption:: malar erythemamalar erythema erythema on finger pulp or toe pulperythema on finger pulp or toe pulp erythema around the nail folderythema around the nail fold

Skin and mucous membrane lesionSkin and mucous membrane lesionss Malar erythema --red macules associated with edeMalar erythema --red macules associated with edema, begin on the malar areas and bridge of the nosema, begin on the malar areas and bridge of the nose, then spread on the ear, tip of the nose, upper eyeli, then spread on the ear, tip of the nose, upper eyelidsds

录象片断

Finger pulp or toe pulp show prunosus macule associFinger pulp or toe pulp show prunosus macule associated with edema, telangiectases or hated with edema, telangiectases or hemorrhagic, atr, atrophy,necrosis. they may also occur on palms, solesophy,necrosis. they may also occur on palms, soles

Skin and mucous membrane lesioSkin and mucous membrane lesionsns Eruptions widespread the body may be vesicle, petEruptions widespread the body may be vesicle, petechia, nodule and livedo reticularis, erythema multiechia, nodule and livedo reticularis, erythema multiform-liked eruptionsform-liked eruptions

Skin and mucous membrane lesioSkin and mucous membrane lesionsns Oral mucosal erythema--erosions, shallow ulceratioOral mucosal erythema--erosions, shallow ulcerations occur commonly on lip, buccal mucosa, the tongns occur commonly on lip, buccal mucosa, the tongue, palatine mucosa,, gums of the teeth .ue, palatine mucosa,, gums of the teeth .

Other internal organs Other internal organs involvementinvolvement

RenalRenal –nephritic or nephrotic—chronic renal i–nephritic or nephrotic—chronic renal insufficiency with azotemiansufficiency with azotemia HeartHeart —myocarditis, pericarditis and endocarditis—myocarditis, pericarditis and endocarditis Pulmo Pulmo —pleural effusions, interstitial lung disease —pleural effusions, interstitial lung disease locomotor system system -Myositis, arthritis-Myositis, arthritis Digestive system Digestive system ——gastroenteritis, peritonitis hephepatitisatitis Nervous systemNervous system —mental disorder, central and per —mental disorder, central and peripheral neuritis or vascular lesionsipheral neuritis or vascular lesions Eye--Retina neural degeneration, conjunctivitis, subhyaloid hemorrhage OthersOthers—Raynaud’s phenomenon, fatigue and fev—Raynaud’s phenomenon, fatigue and fever, er, lymphadenectasis

Laboratory findingsLaboratory findings Hypocytosis Hypocytosis Proteinuria (erythrocyturia \leukocytuProteinuria (erythrocyturia \leukocyturia\ cylindruria)ria\ cylindruria) Elevated sedimentation rate Elevated sedimentation rate Positive rheumatoid factor Positive rheumatoid factor Elevated immunoglobulin Elevated immunoglobulin Low level of serum complement Low level of serum complement

laboratory findingslaboratory findings injured nuclearinjured nuclear

Anti-nucleoprotein antibody

Attract PMN

groups of cell LE cell

Be phagocytized by PMN

homogeneous body

laboratory findingslaboratory findings Special testSpecial test （（ 11 ）） LE cell( cell smear)LE cell( cell smear)

laboratory findingslaboratory findings Special testSpecial test（（ 22 ）） ANAANA (indirect immuno-fluorescence )(indirect immuno-fluorescence )（（ 33 ）） anti ds-DNA antibodyanti ds-DNA antibody（（ 44 ）） ENA(-SmENA(-Sm 、、 RNPRNP 、、 RoRo 、、 La antibLa antibodiesodies ）） (immuno-blot)(immuno-blot)（（ 55 ）） LBT lupus band testLBT lupus band test (direct immuno-fluorescence )(direct immuno-fluorescence )

histologyhistology There is hyperkeratosis in epidermisThere is hyperkeratosis in epidermis follicular orifice dilat, keratinous plug in plug in itit spinous layer atrophy liquifaction degeneration of basal cells lymphocytes and plasma cell infiltrate in lymphocytes and plasma cell infiltrate in dermis specially around the dermis specially around the appendages of the skin and vascellum and vascellum

DiagnoseDiagnose DLE DLE depended on typicdepended on typical eruptionsal eruptions Discoid erytheDiscoid erythematosusmatosus Adherent scales Adherent scales Horny plugs Horny plugs Centrally atrophy Centrally atrophy DyspigmentationDyspigmentation TelangiectasiaTelangiectasia

1982 Revised American Rheumatism Associati1982 Revised American Rheumatism Association Criteria for the diagnosis of SLEon Criteria for the diagnosis of SLE （（ 11 ））1)Malar erythema2)discoid LE3)Photosesitivity4)Oral ulcer5)Nonerosive arthritis6)Serositis (pericarditis or pleurisy)7)Nephropathy (albuminuria or cellular casts)8)CNS disorder (unexplained seizures or psychosis)

1982 Revised American Rheumatism Associat1982 Revised American Rheumatism Association Criteria for the diagnosis of SLE ion Criteria for the diagnosis of SLE （（ 22 ））9)Hematologic disorder (hemolytic anemia with reticulosis, or leucopenia below 4000 on two occasions, or lymphopenia below 1500 on tow occasions)10)Immunologic disorder: positive LE-cell preparation, or antibody to native DNA or SM antigen, or false positive STS11)Antinuclear antibody in abnormal titer, unexplained A patient may be said to have SLE if four or more criteria are satisfied.

TreatmentTreatment Avoid exposure to strong sunlight, to excessive cAvoid exposure to strong sunlight, to excessive cold, heat, tension , tiredness and infectionold, heat, tension , tiredness and infection For predominant skin lesion, hydroxychloroquinFor predominant skin lesion, hydroxychloroquine, thalidomide, tripterygium wilfordii are recome, thalidomide, tripterygium wilfordii are recommended , topical application of carticosteroidmended , topical application of carticosteroid For patients whose organs involved, carticosteroFor patients whose organs involved, carticosteroid should be administered, and combinations of sid should be administered, and combinations of steroid with immunosuppressive drugs are recomteroid with immunosuppressive drugs are recommended when necessarymended when necessary Others : nonsteroidal anti-inflammatory agents, iOthers : nonsteroidal anti-inflammatory agents, immuno-modulatory drugs and other supportive mmuno-modulatory drugs and other supportive treatmentstreatments