2. What Is Systemic Lupus
Erythematosus?
• Systemic lupus erythematosus (SLE) is a progressive chronic
autoimmune disease that results in inflammation and tissue
damage
• Characterized by flares, spontaneous remission, and relapses
• Highly heterogeneous
• Can affect any part of the body
– Often damages skin, joints, heart, kidneys, lungs, nervous
system
3. ACR (1997) Revised Criteria for
Classification of SLE
Skin Criteria
• Butterfly rash
• Discoid rash
• Sun sensitivity
• Oral ulcerations
Systemic Criteria
• Arthritis
• Serositis
• Kidney disorder
• Neurological disorder
Laboratory Criteria
• Blood abnormalities • Positive ANA blood test
• Immunologic disorder
o Antiphospholipid antibodies, lupus anticoagulant,
anti-DNA, false-positive syphilis test, positive anti-
Sm
4 of 11 needed for a diagnosis
4. SLICC SLE Diagnostic Criteria (2012)
Requirements:>criteria (at least 1 clinical and 1 lab criteria) OR biopsy-proven Lupus
nephritis with positive ANA or Anti-DNA
CLINICAL CRITERIA IMMUNOLOGIC CRITERIA
ACUTE OR SUB ACUTE CUTANEOUS LUPUS ANA
CHRONIC CUTANEOUS LUPUS ANTI-DNA
ORAL ULCERS OR NASAL ULCERS ANTI-SM
NON-SCARRING ALOPECIA ANTIPHOSPHOLIPID ANTIBODY POSITIVITY,
AS DETERMINED BY POSITIVE TEST FOR
LUPUS ANTICOAGULANT
SYNOVITIS INVOLVING 2 OR MORE JOINTS
SEROSITIS
RENAL
DIRECT COOMBS’ TEST (IN THE ABSENCE OF
HEMOLYTIC ANEMIA
NEUROLOGIC
HEMOLYTIC ANEMIA
LEUKOPENIA (<4000/MM3) OR
LYMPHOPENIA (<1000/MM3)
THROMBOCYTOPENIA (<100,000/MM3)
5. Diagnosis
• No single test can determine whether a person has lupus, but several
laboratory tests may help make a diagnosis
• Diagnostic Tests
– Antinuclear antibody (ANA) test
– Autoantibodies: anti-DNA, anti-Sm, anti-RNP, anti-Ro (SSA), and anti-
La (SSB)
– Anticardiolipin antibody
– Antiphospholipid antibody
– Skin biopsy
– Kidney biopsy
6. CUTANEOUS LUPUS ERYTHEMATOSUS
• CLE is Lupus affecting the skin.
• Body’s immune system attacks healthy skin.
• Three main sub types are:
a. Acute Cutaneous Lupus (ACLE).
b. Sub Acute Cutaneous Lupus (SCLE).
c. Chronic Cutaneous Lupus (CCLE).
7. CUTANEOUS LUPUS ERYTHEMATOSUS
ACLE SCLE CCLE
• Acute clinical course • Sub acute clinical
course
• Chronic clinical course
• Rapid development of
Cutaneous
manifestation
• Development of
Cutaneous
manifestation Over
several weeks
• Development of
Cutaneous
manifestation Over
several months
• 90% associated with
systemic involvement
• 20-30% associated
with systemic
involvement
• Less than 5%
associated with
systemic involvement
8. Acute cutaneous lupus Erythematous
• There are two Forms of ACLE
i. Localize Form
ii. Generalize Form
9. LOCALIZE FORM
• Malar or butterfly rash that covers cheeks and nasal bridge.
• Forehead and anterior neck may included.
• Nasolibial folds are spared.
• Rash triggered by sunlight, last days to weeks.
• Reddish purple discolourization with mild edema and or papule are
common.
• d/ds acne rosescea ,seborrheic dematitis, dermatomyocitis which involves
nasolibial folds.
10. GENERALIZE FORM
• Less common than localized form.
• Maculopapular rash, or photosensitive dermitis, above or
below neck on sun exposed areas.
11. Diagnosis
• Skin biopsy
• Basal layer degeneration, edema at upper dermitis,
mononuclear cell infiltration at dermal-epidermal junction,
hyperkeratosis and perivasular inflammation.
• Direct immunoflorence
• Granular deposition of immunoglobins IGM and compliments
C3 at dermal- epidermal junction.
12. Treatment
• Sun protection, Smoking cessation.
• Topical Steroids
• Systemic
• Anti-malarial (chloroquine and hydrochloroquine)
• Oral steroids in severe cases.
13. Subacute cutaneous lupus SCLE
• Rash on sun exposed skin ,upper torso, back, neck and arm,
mid face is usually spared.
• Erythematous, annular, polycystic lesion or may have non
indurated, psoriform, polysquamous appearance.
• 10 to 30% drug induced
• Antihypertensive, thiazide diuretics ,ACE inhibitors, PPIs and
antiepileptics.
• Histopathalogy interface dermitis ,hyperkeratosis, basement
mem thickining, follicular plugging and lymphocyte
infiltration.
• Treatment : Topical steroids , oral antimalarials.
17. Atypical and Rare forms of CLE
• Rare form of ACLE
i. Bullous lupus Erythematous
• Rare form of SCLE
i. Erthrodermic LE
ii. Lupus Erythermatosus gyratus repens
iii. EAC-like LE
18. Atypical and Rare forms of CCLE
• Rare form of CCLE
i. Hypertrophic
ii. Lupus comedonicus
iii. Lupus mastitis
iv. Monogenic LE
v. TEN (Toxic Epidermal Necrolysis)-like LE
vi. EEM(Erythema exsudativum multifome)-like LE
19. Bullous lupus Erythematous
• Disseminated vesicles and crusts on erythematous to
clinically normal skin.
• Accompanied by residual hyperpigmentation and milia.
20. Erthrodermic LE
• Rare form of subacute cutaneous lupus
occurs after sun exposure. generalized
exfoliative erythroderma.
21. Lupus Erythermatosus gyratus repens
• Very rare varient of SCLE,with chronic
recurrent figurate erythematous annular
plaques.
22. EAC-LIKE LE
• Annular erythematous plaques with trailing
scale similiar to eac erythema annular
centifugum.
23. OTHER FORMS OF ACLE
• TEN like LE.(Toxic Epidermal Necrolysis)
• EEM like LE.(Erythematic Exsudativum )
• Very rare forms of ACLE, associated with drug
reaction.
24. Hypertrophic
• Rare form of CCLE ,on face and extensor
surface of limbs, sole and
palms.yperkeratotic lesion with
congestive margins and central atropy
