CUTANEOUS LUPUS

ERYTHEMATOSUS
SGD B3
CUTANEOUS LUPUS
ERYTHEMATOSUS
 Introduction
 Etiology
 Pathogenesis
 Clinical Feature
 Diagnosis and Differential Diagnosis
 Therapy
 Conclusion
INTRODUCTION
INTRODUCTION
 The term “lupus” originated in ancient
Greece
 Disease with a broad spectrum of
cutaneous and systemic manifestations.
 In 1845, Ferdinand von Hebra (1816–
1880) described butterfly erythema of
the face as “seborrhoea congestiva”, but
it was not until 1866 that the disease
was named “lupus erythematosus”
Cutaneous Lupus Erythematosus (CLE): a
chronic inflammatory autoimmune
disease with a broad spectrum of
cutaneous clinical manifestations (seen
in 72-85 % of patient with SLE)
It divided into 3 subtypes: Acute CLE,
Subacute CLE, and Chronic CLE
 appears more common among Afro-
Americans and Asians race
 female: male ratio = 9:1
 age ranges 15-64 years = 4:1 within the
other ranges
 12% CLE  SLE progression being 8.2
years
 DLE  SLE progression 2 yrs
Contents

ETIOLOGY
ETIOLOGY
 HOST FACTOR

1. Interactions between genetic, infective, and

hormonal factors  immunoregulation
(association between HLA-A1, -B8, -DR3, -A3,
-B7, -DR2 and C4A halotypes, also the IL-1 and
TNF-α)  pathogenesis of CLE

2. High level of estrogen and progesterone

promote humoral autoreactivity

3. Pregnancy and oral contraceptive (unknown

explanation)
 ENVIRONMENTAL FACTORS

1. UV light self-immunity, loss of tolerance apoptosis of

keratinocytes
- UVB radiation displace autoantigens

2. Drugs  T-cell DNA hypomethylation altered repair of

DNA  decreased amount of methylation of DNA 
increased biologic autoreactivity of lymphocyte
- e.g. procainamide, hydralazine, and isoniazide

3. Infectous agent  controversial??

4. Tobacco  smokers >> risk than non-smoker

Content

PATHOGENESIS
PATHOGENESIS of CLE
 Interaction between host & environmental factor
 This lead into of self & triggering autoimmunity
A. Host factor:
1) Susceptible gene:-
i. TNF
 increase TNF-α, increase apoptosis process
ii. Complement:-
 development photosensitivity
iii. HLA:-
 shaping T-cell repertoire
 autoantibodies
2) Hormone:
i. estrogen
ii. androgen
PATHOGENESIS of CLE
B. Environmental factor:
1) UV radiation
 cause apoptosis of keratinocytes
 displace autoantigen:
- Ro/ss-A
- La/ss-B
- calreticulin
 effect the immunoregulatory cell
2) Tobacco
 complex immunomodulatory effect
3) Drug
 provocative agent – altered repair of DNA
4) Viral infection
 rubella
 cytomegalovirus
Content

CLINICAL FEATURES
CLINICAL FEATURE

 Acute CLE (ACLE)

 Subacute CLE (SCLE)
 Chronic CLE (CCLE)
 Discoid LE (DLE)
 LE Profundus (LEP)

 Chilblain LE (CHLE)
ACLE
ACLE
 Malar / butterfly rash
in SLE
SCLE
SCLE
 Annular subtype with
polycyclic
confluenced in sun-
exposed area
CCLE - DLE
CCLE - DLE
 Erythema,
hyperkeratosis, and
scarring alopecia
CCLE - LEP
CCLE - CHLE
CCLE - CHLE
 Livid infiltration with
central crusting
Content

DIAGNOSIS AND
DIFFERENTIAL DIAGNOSIS
Anamnesis
 History of photosensitivity or malar rash common but
not necessary
 Many patients have systemic complaints, such as
fevers, fatigue, and malaise.
 Many patients complain of joint pain, Raynaud
phenomenon, or alopecia.
 Chest pain from pericarditis or pleural effusions may
be present.
 Raynaud phenomenon can be associated with lupus.
 Is he/she taking hydralazine or procainamide
(common causes of drug-induced lupus
erythematosus)
Physical Examination
 According to diagnostic criteria

 Presence of systemic disease (fever, malaise,

arthralgias, seizures, psychosis, pleural chest pain,
and photosensitivity)

 signs of myolitis, arthritis, dyspnea, pleuritis,

pericarditis, neurologic, and neuropsychiatric disorder
Laboratory testing
 Cytopenia: Anemia, leukopenia (<4,000/mm3), lymphopenia
(<1,500/mm3), or thrombocytopenia (<100,000/mm3)2

 Nephritis: >0.5 g/d proteinuria or cellular casts

 Serositis: Pleuritis or pericarditis

 Immunologic disorder: Antibodies to double-stranded DNA, Smith

nuclear antigen, LE-cell preparation, false-positive serologic test
for syphilis, or antiphospholipid antibodies (anticardiolipin
antibodies or positive lupus anticoagulation test)2

 Positive ANA
Found in >95% patients with SLE, but positive ANA can occur in
many diseases (and in 10–20% of normal population)
 Almost always positive DIF
Revealing deposits of IgG and IgM, complement components (C3
and C1q)2
Differential Diagnosis
 Systemic juvenile-onset rheumatoid arthritis
 Oncologic disease (leukemia, lymphoma)
 Other vasculitic disorders
 Dermatomyositis
 Fibromyalgia
 Drug-induced lupus
 Pitfalls
Content

THERAPY
 Mainly treated by photoprotection,
topical steroids and hydroxycloroquine.
 Antimalarias is use to treat severe case
to achieve optimal result with minimal
toxicity.
 Cytotoxic agents restricted to patients
with organ threatening systemic disease.
Surgical Care and Consultations
Surgical Care
 Surgical approaches rarely are needed in SCLE patients

Consultations
 Consultations with the following specialists may be
helpful:
 Rheumatologist - When joints are involved
 Nephrologist - When renal involvement is present
 Neurologist - When CNS disease is present
 Internal medicine specialist - For systemic involvement
Diet and Activity
Diet
 No special diet is required with SCLE.

Activity
 Since SCLE is exacerbated by sunlight or other UV
light exposure, advise patients to take precautions.
One precaution is to discourage exposure to sunlight
between the hours of 10 am and 4 pm.
Medications

 Antimalarials have immunomodulatory effects that may

improve symptoms of the disease. Hydroxychloroquine
is DOC for systemic therapy of SCLE.
Chloroquine is second line.
 Leprostatic agents may have immunomodulatory
effects.
 Immunomodulators are effective in the treatment of
diseases with autoimmune etiology.
 Gold compounds may regulate immune cell function.
 Retinoids play a role in cell growth and differentiation
Content

PROGNOSIS
 CLE is less severe course and a better
prognosis than SLE
 Signs of nephropathy, elevated
antinuclear antigen titers, and arthralgias
may serve as predictors for transition
into SLE
 CLE  SLE within 8.2 yrs
CONCLUSION
CONCLUSION
 CLE  chronic inflammatory autoimmune
disease divided into nonspecific CLE which is
associated with the SLE and specific CLE.
 3 subtypes of CLE  acute CLE, subacute CLE and
chronic CLE (discoid, profundus, chilblain)
 Etiology: host ( genetics & hormones) &
environmental factors (UV light, drugs, viruses and
tobacco )
 Pathogenesis: Interaction between host and
environmental factors. It involves 3 phases:
 Initiation
 amplification
 target damage
CONCLUSION cont.
 Each subtype manifest different clinical features and the diagnosis
is made from anamnesis, physical examination and labaratory
results.
 DD: Systemic juvenile-onset rheumatoid arthritis
Oncologic disease (leukemia, lymphoma)
Othervasculitic disorders
Dermatomyositis
Fibromyalgia
Drug-induced lupus
Pitfalls
 Therapy: photoprotection, topical steroids, hydroxycloquine,
antimalaria (in severe and refractory case), cytotoxic agents ( in
patients with organ threatening systemic disease) systemic
corticosteroids (for treatment active SLE with heart , kidney ,
hematologic , and central nervous system involvement).
 Education to reduce/relieve anxiety and to recruit the patient as an
active participant in the treatment regimen