L U P U S E R YT H E M A T O U S

PHOTODERRMATOSES
WITH

THOMAS RUENGER

MUHAMMAD KHAWAR NAZIR

11-10-2009
LUPUS ERYTHEMATOSUS

 Multisystem disorder that prominently

affects the skin

 Cutaneous lesions are a source of

disability and, on many occasions, an
indicator of internal disease
LUPUS ERYTHEMATOSUS
 Lupus can cause disease of the skin, heart, lungs,
kidneys, joints, and nervous system

 When only the skin is involved, the condition is

called Discoid Lupus

 When internal organs are involved, the condition is

called systemic lupus erythematosus(SLE)

 Up to 10% of persons with discoid lupus (lupus limited

to the skin) eventually develop the systemic form of
lupus (SLE).
 ACUTE CUTANEOUS LUPUS

 Acute cutaneous lupus involves primarily

the epidermis and upper dermis and is
usually associated with systemic disease

▪  Subacute cutaneous lupus involves

primarily the epidermis and upper dermis
and is associated with anti-Ro
autoantibodies and photosensitivity; the
majority of patients do not have significant
systemic disease
CHRONIC CUTANEOUS LUPUS

▪    DISCOID LUPUS involve the epidermis, upper and lower

dermis, and adnexal structures, and they can scar; the
majority of patients do not have significant systemic disease

▪  Lupus Erythematosus Tumidus involves the dermis but there

is no prominent adnexal involvement

▪   Lupus Panniculitis involves the subcutaneous tissue and

may result in disfiguring depressed scars

 LUPUS PROFUNDUS ; lupus panniculitis with overlying

Discoid lesions
Relationship of ACLE to  Relationship of cutaneous
lupus erythematosus to
CCLE and SCLE systemic disease
 Ultraviolet Radiation

 Most important environmental factor in the induction

phase of SLE and especially of LE-specific skin
disease

 Early studies demonstrated that CLE lesions could be

provoked in the clinically normal skin of patients with
both SLE and CLE by repeated delivery of high
doses of UVB radiation to the same test site

 More recent studies argue that UVA radiation can

also induce CLE lesions
 Ultraviolet Radiation
 UV light likely leads to self-immunity and loss of tolerance because it
causes apoptosis of keratinocytes, which in turn, makes previously cryptic
peptides available for immunosurveillance

 UVB radiation has been shown to displace autoantigens such as

Ro/SS-A and related autoantigens, La/SS-B, and calreticulin, from
their normal locations inside epidermal keratinocytes to the cell surface

 Anti-SS-A/Ro Robert-antigen or soluble substance A nuclear antigen

 Anti-SS-B/La Lane-antigen or soluble substance B nuclear antigen

 UV light causes an exaggerated release of immune mediators in

patients with SLE
 Ultraviolet Radiation

 Recent work by Meller and colleagues shows that UVB irradiation

induces the release of CCL27 (cutaneous T cell-attracting
chemokine), which upregulates the expression of chemokines that
activate autoreactive T cells and interferon- (IFN- ), producing
dendritic cells (DCs), which likely play a central role in lupus
pathogenesis

 UV light may additionally have an early critical role in the induction

phase by directly altering cellular DNA

 UV light may affect immunoregulatory cells, which normally help

suppress "abnormal" patterns of cutaneous inflammation.
 Approach to the patient with skin lesions suspicious for CLE
Eleven criteria have been established for the diagnosis of SLE:

 Malar (over the cheeks of the face) "butterfly" rash

 Discoid skin rash: patchy redness that can cause scarring
 Photosensitivity: skin rash in reaction to sunlight exposure
 Mucus membrane ulcers: ulceration of the lining of the
mouth, nose or throat
 Arthritis: 2 or more swollen, tender joints of the extremities
 Pleuritis/pericarditis: inflammation of the lining tissue around
the heart or lungs, usually associated with chest pain with
breathing
 Kidney abnormalities: abnormal amounts of urine protein or
cellular elements
 Brain irritation: manifested by seizures (convulsions) and/or
psychosis
 Blood count abnormalities: low counts of white or red blood
cells, or platelets
 Immunologic disorder: abnormal immune tests include anti-
DNA or anti-Sm (Smith) antibodies, falsely positive blood test
for syphilis, anticardiolipin antibodies, lupus anticoagulant,
or positive LE prep test
 Antinuclear antibody: positive ANA antibody testing
Acute Cutaneous Lupus Erythematosus

 Bilateral malar erythema (butterfly rash). The

lesions tend to be transient, follow sun exposure,
and resolve without scarring (but sometimes with
dyspigmentation)

 An association with anti-dsDNA antibodies and

lupus nephritis has been proposed and is plausible,
although some patients with a malar rash have
neither anti-dsDNA antibodies nor lupus nephritis

 Patients presenting with this type of eruption must

be evaluated carefully for evidence of internal
disease.
Subacute Cutaneous Lupus Erythematosus

 Typically photosensitive, with lesions confined to

sun-exposed skin

 Non Scarring

 Although the relationship of SCLE lesions to sun is

striking, it is also notable that the midfacial skin is
usually spared, while the sides of the face, V of
the neck, and extensor aspects of the upper
extremities are commonly involved

 In some instances, lesions of SCLE have

appeared in patients receiving certain
medications, in particular hydrochlorothiazide and
terbinafine, but also calcium channel blockers

 Regular association with the anti-Ro

autoantibody
Chronic Cutaneous Lupus Erythematosus
 DISCOID LUPUS
 Most common type of cutaneous lupus

 Discoid lesions are found most often on the face,
scalp and ears

 Unusual for discoid lesions to be present below the

neck without lesions also being present above the neck

 Discoid lesions have the potential for scarring, and,

with time, a substantial proportion of patients develop
disfiguring scarring
 DLE
 Intense inflammatory
infiltrate, prominent both in
the superficial and deep
dermis and surrounding
adnexal structures, is seen
in discoid lesions

 Marked hyperkeratosis with

follicular plugging is also
present. 
 Immunopathology of LE–specific skin disease
Direct immunofluorescence examination of a Discoid Lupus erythematosus
lesional skin biopsy showing a continuous band of granular fluorescence at the
dermal-epidermal junction as a result of staining with fluorescein isothiocyanate-
conjugated goat anti-immunoglobulin G.
Therapy of cutaneous lupus

Local therapy

Sun protection
Topical and intralesional corticosteroids
Topical calcineurin inhibitors
Topical retinoids
Systemic antimalarial therapy

Hydroxychloroquine (200 mg po qd–bid in adults;

up to 6.5 mg/kg ideal body weight/day)

Chloroquine (125–250 po qd in adults; up to 3.5–

4 mg/kg ideal body weight/day)

Quinacrine (100 mg po qd)

Combination of hydroxychloroquine or
chloroquine and quinacrine (2)
 Systemic therapy for antimalarial-resistant
cutaneous disease
Retinoids (e.g. acetretin, isotretinoin)

Thalidomide (50–100 mg po qd for clearing and, if necessary, 25–50 mg po

qd–twice
weekly for maintenance)
Gold

Dapsone (primarily for bullous eruption of SLE)

Clofazimine

Sulfasalazine

Immunosuppressive agents (e.g., azathioprine)

Systemic corticosteroids

Immune response modifiers (e.g., rituximab, anti-BlyS, CTLA4-Ig, anti-IL-6,

anti-IL
 Adjunctive Therapy

Sun Protection
Sun protection is a vital part of
therapy for many patients
because the sun exacerbates
or initiates their skin lesions
 Sun Protection
 sun protection is important for cancer
prevention, particularly in hypopigmented skin
or in chronic discoid lesions, where the risk of
skin cancer development may be higher

 Cancer prevention is also essential for patients

who are on immunosuppressive therapy
 Sun Protection
 It has been reported that sun exposure can
exacerbate systemic disease in patients who
have SLE

 Therefore, there are a variety of reasons why

sun protection should be emphasized, even in
persons whose skin lesions are not induced or
exacerbated by sun exposure.
 Sun Protection
 Advise patients to avoid direct sun exposure

 Wear tightly woven clothing and broad-brimmed hats

 Regularly use broad-spectrum, water-resistant

sunscreens [SPF 30 with an efficient UVA blocking
agent such as a photostabilized form of avobenzone
(Parsol 1789), micronized titanium dioxide, micronized
zinc oxide, or Mexoryl SX]
 Sun Protection
 UV-blocking films should be applied to home and
automobile windows, and acrylic diffusion shields
should be placed over fluorescent lighting

 Corrective camouflage cosmetics such as

Dermablend® and Covermark® offer the dual benefit
of being highly effective physical sunscreens as well as
aesthetically pleasing cosmetic masking agents


 Prevention

 Predicting and preventing the initial clinical

manifestation of LE, whether it is skin disease or
systemic, is not feasible at this time

 However, as many LE patients exhibit worsening of

their skin disease activity with UV light exposure,
physical protection from sunlight and artificial sources
of UV light as well as the regular use of broad-
spectrum sunscreens having a SPF of 30 or greater
should be encouraged.
 Patient Education

 Education on the optimal use of

sunscreens and protective clothing and
effective approaches to sun avoidance
is important for most patients with lupus
TH A N K Y O U