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