02. Lupus Erythematosus-1. dermatological condition

Lupus

1. Lupus erythematosusLupus erythematosus
Dr. NgalamikaDr. Ngalamika

2. •Autoimmune disease
•May manifest as a systemic disease or in
purely cutaneous forms
•Has chronic relapsing and remitting course
•Most commonly affects the skin, kidneys,
joints, blood vessels, liver, lungs, and
nervous system

3. EtiologyEtiology
•Genetic polypmorphisms: increased
expression of pro-inflammatory cytokines
•Epigenetics
•UVA & UVB: upregulate antigen
expression and cytokines leading to
photosensitivity & UV-induced flares of
systemic disease
•Reduced T-Suppressor cell function

4. •Over production of antibodies by B cells
•Reduced clearance of immune complexes
by reticuloendothelial system
•Abnormal apoptosis or reduced clearance
of apoptotic cells lead to increased
exposure to nuclear antigens

5. Possible triggersPossible triggers
•Sex Hormones
•Infections
•Drugs
•Cigarette smoking: associated with
increased disease activity
•Dietary factors: excess calories

8. Cutaneous lupus subsetsCutaneous lupus subsets
1.Acute cutaneous lupus erythematosus
(ACLE)
2.Subacute cutaneous lupus erytematosus
(SCLE)
3.Chronic cutaneous lupus erythematosus
(CCLE)
4.Systemic lupus erythematosus
5.Neonatal lupus erythematosus

9. Acute cutaneous Lupus (ACLE)Acute cutaneous Lupus (ACLE)
•Highly associated with
SLE (~100%)
•Photosensitive malar rash
is characteristic
•Occurs in 20% to 60% of
SLE patients
•Lasts from days to weeks
•Presence of violaceous
erythematous patches or
plaques over the malar
eminences, may involve
entire face with sparing of
nasolabial folds

10. •May heal with dyspigmentation or
poikiloderma, but does not scar
•Often painful or pruritic
•Associated with sun exposure

11. •May be generalized
and characterized by
erythematous
macular or papular
scaling with or without
edema

12. Subacute cutaneous lupus Subacute cutaneous lupus
erythematosus (SCLE)erythematosus (SCLE)
•Associated with HLA-B8, HLA-DR3, HLA-
DRw52, and HLA-DQ1
•Usually occurs in caucasian females; seen
in 9% to 27% of patients with SLE

13. Clinical featuresClinical features
•Begins as
erythematous papules
or plaques
•Annular lesions or
scaling plaques,
usually on sun
exposed areas but
may be generalized
•Knuckles usually
spared when lesions
occur on the hands
•Has a waxing and
waning course
•Heals with
hypopigmentation
•50% meet ACR
criteria for SLE
•Only 10% to 20% of
patients develop SLE
•Can be drug induced

15. treatmenttreatment
•Sun-protection
•Hydroxychloroquine, other antimalarials,
thalidomide, azathioprine, acitretin,
Mycophenolate mofetil, dapsone

16. Chronic cutaneous lupus Chronic cutaneous lupus
erythematosus (Discoid LE)erythematosus (Discoid LE)
•Chronic, scarring and photosensitive
disease
•Occurs in 15% to 30% of patients wit SLE,
5% of patients with DLE progress to SLE
•Female to male ratio of 3:1

17. Clinical featuresClinical features
•Begins as
erythematous papules
or plaques
•Progresses to
plaques with follicular
plugging, scale,
central
hypopigmentation,
and peripheral
hyperpigmentation
•Can be localized or
widespread
•Lesions resolve wit
permanent scarring
•Less photosensitive
than other forms

19. treatmenttreatment
•Corticosteroids (topical & intralesional)
•Antimalarials
•Thalidomide
•Azathioprine
•MMF
•MTX

20. SLESLE
•Multisystem autoimmune disorder
•Found commonly in women of child
bearing age
•Associated with HLA DR-2 and DR-3

21. Diagnostic criteria for SLE Diagnostic criteria for SLE
(ACR)(ACR)
1.Malar rash
2.Discoid rash
3.Oral ulcers
4.photosensitivity
5. Arthritis
6. Serositis
7. Renal involvement
8. Hematologic disease
9. Neurologic disorders
10. ANA
11. Immunologic
disorders

22. Other associated manifestations Other associated manifestations
•Constitutional
symptoms (fever,
malaise, waight
changes)
•Vasculitis
•Urticaria
•Lupus panniculitis
•Livedo reticularis
•Raynaud’s
phenomenon
•Alopecia
•Arthralgias
•Headache
•TIAs, stroke
•Myelopathy
•Abdominal pain
•Recurrent
spontaneous
abortions
(antiphospholipid
antibodies)

23. Laboratory testsLaboratory tests
•ANA (positive in 95%
of SLE cases)
•Anti-Smith ab (highly
specific for SLE)
•Anti-dsDNA (indicates
high risk of renal
disease)
•Anti-nRNP (high titres
in connective tissue
disease, low titres in
SLE)
•Anti-La ab (common
in SS & SCLE)
•Anti-Ro ab (SLE,
SCLE, SS, neonatal
LE)
•Serum complement:
Low levels in active
disease

24. Lab tests, Lab tests, continued…continued…
•Lupus band test: DIF
•Anti-ssDNA: sensitive
but not specific
•Anti-phospholipid
antibodies: may occur
occur in association
with lupus and oter
connective tissue
disease, or as a
solitary event

25. Other lab testsOther lab tests
•Raised ESR or CRP
•FBC: hemolytic
anemia, leukopenia,
thrombocytopenia
•Urinalysis:
proteinuria, cellular
casts
•MRI: stroke, vasculitis
•CT chest
•Renal biopsy
•Skin biopsy

26. TreatmentTreatment
•Prednisone
•Azathioprine
•MMF
•Cyclophosphamide
•Hydroxychloroquine
•MTX

27. DRUG-INDUCED LUPUS DRUG-INDUCED LUPUS
ERYTHEMATOSUSERYTHEMATOSUS
•Autoantibody profile varies from classic
SLE; no renal or CNS involvement; malar
and discoid lesions uncommon
•Commonly associated with antihistone
antibodies ANAs
•Associated with HLA-DR4
•Slow acetylators have a higher DILE risk
•

28. Etiologic agentsEtiologic agents
•Antiarrhythmics:
procainamide,
quinidine
•Antibiotics:
minocycline
•Antifungal:
voriconazole,
griseofulvin
•Anticonvulsants:
phenytoin,
carbamazipine
•Hormonal therapy:
OCPs
•Antihypertensives:
hydralazine
•Anti-inflammatory: D-
penicillamine
•Antipsycotics:
chlorpromazine

29. Neonatal LENeonatal LE
•Caused by mother’s antibodies (Anti-
Ro:95%, anti-La) in the fetus
•Most affected infants are girls
•Skin lesions develop during the first weeks
of life

30. Clinical featuresClinical features
•Annular, erythematous, macules and
plaques on head and extremities
•Periocular involvement may be prominent
(racoon eyes)
•Photosensitivity
•High risk of congenital heart block (15-
30%)

31. Laboratory testsLaboratory tests
•ANA
•Maternal & neonatal anti-Ro, anti-La, anti-
U1-RNP
•Neonatal ecg/echo
•Immunofluorescence

32. treatmenttreatment
•Photoprotection
•Mild topical steroids
•Heart failure: pacemaker

33. Other forms of LupusOther forms of Lupus
•Chilblain LE
•Lupus panniculitis
•Bullous SLE
•Tumid lupus

34. EndEnd