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Lupus erythematosus.

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Presentation on theme: "Lupus erythematosus."— Presentation transcript:

1 Lupus erythematosus

2 Definition Lupus erythematosus LE is classified as connective tissue disease. It occurs predominantly in females in the reproductive years. Multiple abnormal immune responses are present. It may involved any organ of the body and is manifested in many forms. It may be life-threatening when it becomes severe.

3 Etiology suggestion : the findings of 100 LE family surveys :
genetic factors the findings of 100 LE family surveys : Family history positive : % The prevalence of SLE in first-degree relatives is % The degree of heritability was 56 % In addition, the concordance rate in monozygotic twins comes to over 50% suggestion : LE is multifactorial inheritance Environmental influence high

4 Etiology Enviornmental influence
Virus infection-- other bacterial infections are seldom Physical factor -- sunlight ,coolness, wetness, ray Emotional factor ---stress, worry Endocrine factor –high estrogen ,prolactin drugs -- more than 20 drugs such as penicillin, phenytoin may induce symptomatic LE

5 Pathogenesis Environmental influence (infection, sunlight, drug)
Genetic factor Immuno-modulatory function disorder Auto-antigen formed Sensitivitied T- lymphocytes Various auto antibody Immune-complexes precipitation Blood cell injured Organs injured Alterations in organs

6 Clinical manifestation
Discoid LE ←→★ Systemic LE

7 Discoid lupus erythematosus, DLE
Skin lesions are predominantly manifested Eruptions may occur singly or be multiple localized DLE Lesions localized on face, dorsa of the hand generalized DLE Lesions widespread on trunk ,extremities

8 Skin lesions in discoid LE
Red macules or papules associated with edema→→ discoid eyrthematosus with adherent scales, horny plugs—-centrally atrophy, dyspigmentation, telangiectasia


10 录象片断

11 skin lesions in discoid LE Favorite sites are malar areas, bridge of nose, lower lip, ear, scalp and dorsa of the hand

12 录象片断

13 Skin lesions in discoid LE atrophy in the scalp → false alopecia areata lesions on low lip may be eroded

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

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

16 Skin and mucous membrane lesions
Malar erythema --red macules associated with edema, begin on the malar areas and bridge of the nose, then spread on the ear, tip of the nose, upper eyelids

17 录象片断

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

19 Skin and mucous membrane lesions
Eruptions widespread the body may be vesicle, petechia, nodule and livedo reticularis, erythema multiform-liked eruptions

20 Skin and mucous membrane lesions
Oral mucosal erythema--erosions, shallow ulcerations occur commonly on lip, buccal mucosa, the tongue, palatine mucosa,, gums of the teeth .

21 Other internal organs involvement
Renal –nephritic or nephrotic—chronic renal insufficiency with azotemia Heart —myocarditis, pericarditis and endocarditis Pulmo —pleural effusions, interstitial lung disease locomotor system -Myositis, arthritis Digestive system —gastroenteritis, peritonitis hepatitis Nervous system —mental disorder, central and peripheral neuritis or vascular lesions Eye--Retina neural degeneration, conjunctivitis, subhyaloid hemorrhage Others—Raynaud’s phenomenon, fatigue and fever, lymphadenectasis

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

23 laboratory findings injured nuclear Anti-nucleoprotein antibody
homogeneous body Be phagocytized by PMN Attract PMN groups of cell LE cell

24 laboratory findings Special test(1)LE cell( cell smear)

25 laboratory findings Special test (2)ANA
(indirect immuno-fluorescence ) (3)anti ds-DNA antibody (4)ENA(-Sm、RNP、Ro、La antibodies) (immuno-blot) (5)LBT lupus band test (direct immuno-fluorescence )


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

28 Diagnose DLE depended on typical eruptions Discoid erythematosus
Adherent scales Horny plugs Centrally atrophy Dyspigmentation Telangiectasia

29 1982 Revised American Rheumatism Association Criteria for the diagnosis of SLE(1)
1)Malar erythema 2)discoid LE 3)Photosesitivity 4)Oral ulcer 5)Nonerosive arthritis 6)Serositis (pericarditis or pleurisy) 7)Nephropathy (albuminuria or cellular casts) 8)CNS disorder (unexplained seizures or psychosis)

30 1982 Revised American Rheumatism Association Criteria for the diagnosis of SLE (2)
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 STS 11)Antinuclear antibody in abnormal titer, unexplained A patient may be said to have SLE if four or more criteria are satisfied.

31 Treatment Avoid exposure to strong sunlight, to excessive cold, heat, tension , tiredness and infection For predominant skin lesion, hydroxychloroquine, thalidomide, tripterygium wilfordii are recommended , topical application of carticosteroid For patients whose organs involved, carticosteroid should be administered, and combinations of steroid with immunosuppressive drugs are recommended when necessary Others : nonsteroidal anti-inflammatory agents, immuno-modulatory drugs and other supportive treatments

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