CUTANEOUS LUPUS ERYTHEMATOUS

1. CUTANEOUS LUPUS ERYTHEMATOUS
Tayyaba Mumtaz
PG#2

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.

14. Subacute cutaneous lupus SCLE

15. Chronic cutaneous lupus CCLE
• Most common is discoid lupus,
• Lesion classically on face, scalp, ears can also effect lips, nasal
mucosa, conjunctiva and genitilis.
• Triggers sun exposure, trauma, cold exposure, infection,
dermatitis, thermal burns.
• Coin shaped erythematous plaque, follicular hyperkeratosis
and plugging , high potential for scarring.
• Treatment topical steroids, topical calcineris,
antimalarials,methotrexate,sytemic retinoid, thalidomide,
dapsone, azothioprine.

16. Chronic cutaneous lupus CCLE

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

25. Lupus Comedonicus
• Erythematous to brown infiltrated plaques
with open comedones .

26. LUPUS MASTITIS
• Breast involvement of lupus prefundus,
chronic course with remission and flares.

27. MONOGENIC LE
• Rare variant with single gene mutation, occurs
in children manifest in CNS, kidney, joint and
skin.

28. LINEAR LE
Rare form CCLE, in children, especially on face and
limbs.

29. THANK YOU