This document discusses three types of cutaneous lupus erythematosus: systemic lupus erythematosus (SLE), subacute cutaneous lupus erythematosus (SCLE), and discoid lupus erythematosus (DLE). SLE is an autoimmune disorder characterized by autoantibodies against nuclear DNA. SCLE is a variant of lupus characterized by anti-Ro antibodies. DLE causes erythematous plaques and scarring, often affecting the face and ears. Treatment involves photoprotection, topical steroids, hydroxychloroquine and occasionally oral steroids depending on the severity of symptoms for each condition.

1. 1- SYSTEMIC LUPUS ERYTHEMATOSUS
2- SUBACUTE CUTANEOUS LUPUS
ERYTHEMATOSUS
3- DISCOID LUPUS ERYTHEMATOSUS
Prepared By :- Dr Monther Fadel Nagi
Dermatology Resident

2. Systemic Lupus Erythematosus

3. Pathogenesis
•
Systemic lupus erythematosus (SLE), sometimes
•
referred to simply as lupus, is an autoimmune disorder
•
characterized by autoantibodies against various
•
components of nuclear DNA. Subtypes of these
antibodies include anti–double-stranded DNA antibodies
•
(anti-dsDNA), anti-Smith antibodies (anti-Sm), and
•
antihistone antibodies. The latter are usually associated
•
with drug-induced lupus

4. Drugs Induced SLE
•
Commonly Implicated Drugs in Drug-Induced
•
Systemic Lupus Erythematosus
•
• Hydralazine
•
• Isoniazid
•
• Minocycline
•
• Phenytoin
•
• Procainamide
•
• Quinidine

5. Clinical Features
•
• SLE usually affects middle-aged adults, with women
•
being affected more often than men.
•
• Malar erythema of the face (butterfly rash) is the
•
classic cutaneous finding .
•
• Macular erythema, erythematous papules, or even
•
urticarial lesions can be observed on photoexposed
•
skin of the face, neck, back, chest, arms, and hands.
•
• When the hands are involved, lesions tend to involve
•
the interphalangeal skin, in contrast to dermatomyositis,
•
which tends to involve skin over the joints.
•
• Involvement of the oral mucosa is not uncommon

6. Clinical Features
•
• Rarely, vesicles or blisters may form (bullous systemic
•
lupus erythematosus).
•
• About 5% to 10% of patients with SLE may demonstrate
•
discoid lupus erythematosus lesions.
•
• Diffuse nonscarring alopecia can occur, often with
•
broken-off hairs (so-called lupus hair).
•
• Constitutional symptoms can include fever and malaise.
•
• Systemic complications include arthralgias and
myalgias, renal disease (≈50%), lymphoreticular
•
complications, cardiac complications, and involvement
•
of the bone marrow or central nervous system.

7. Clinical Diagnosis
•
• The appearance of a photodistributed erythema
•
with systemic symptoms should prompt an evaluation
•
for SLE.
•
• An ANA laboratory test should always be done, with
nearly all cases showing ANA positivity, often in high titer.
•
• Additional laboratory studies include a CBC,
determination of the erythrocyte sedimentation rate (ESR)
•
and C-reactive protein (CRP) level (useful to monitor
•
disease activity), urinalysis, renal function tests, liver
•
function tests, and total complement activity (CH50;
•
depressed values correlate with renal disease).

8. Clinical Diagnosis
•
• A punch biopsy (3 or 4 mm) of lesional skin that
•
includes subcutaneous fat will usually be diagnostic
•
of SLE, although it is often not possible to exclude
•
dermatomyositis or other forms of lupus erythematosus
•
completely without clinical details.
•
• A punch biopsy (3 or 4 mm) from photodistributed
•
nonlesional skin for direct immunofluorescence will
•
highlight a lupus band in 60% to 80% of cases.

9. Treatment
•
• Photoprotection (e.g., hats, clothing, sun avoidance,
•
UVA sunscreen) is necessary.
•
• Drugs that may induce SLE (see box) must be
withdrawn or substituted
•
• Topical corticosteroids may be tailored to the site and
•
severity of disease. In general, truncal lesions often
•
require moderate to ultrapotent corticosteroids.

10. Treatment
•
• Oral prednisone (starting at 40–60 mg/day) may be
•
used, with a taper based on the response.
•
• Immunosuppressive drugs to use in a steroid-sparing
•
regimen usually include mycophenolate mofetil,
•
cyclosporine, azathioprine, cyclophosphamide, and
•
rituximab.
•
• Hydroxychloroquine, chloroquine, or quinacrine
•
may be used to manage cutaneous disease.

11. Subacute Cutaneous Lupus
Erythematosus

12. Pathogenesis
•
Subacute cutaneous lupus erythematosus (SCLE) is
•
a largely cutaneous variant of lupus. As the name
•
implies, the clinical course is usually less severe and
•
less acute than SLE. SCLE is characterized by the
•
presence of anti-Ro (anti-SSA) antibodies and, less
•
often, by anti-La (and SSB) antibodies. Less often,
•
SCLE appears to be triggered by some medications

13. Drugs Induced SCLE
•
Commonly Implicated Drugs in Drug-Induced
•
Subacute Cutaneous Lupus Erythematosus
•
• Diltiazem
•
• Hydrochlorothiazide
•
• Leflunomide
•
• Oxprenolol
•
• Terbinafine
•
• Ranitidine
•
• Verapamil

14. Clinical Features
•
• Middle-aged to elderly women are usually affected.
•
• In SCLE, the photodistributed lesions usually affect
•
the face, upper chest, arms, and upper back.
•
• The malar erythema of SCLE may be clinically
indistinguishable from that of SLE.
•
• Erythematous lesions of the arms and trunk may
•
demonstrate annular lesions or erythematous scaly
plaques that may resemble psoriasis. Not all lesions
manifest scale.

15. Clinical Features
•
• The scarring and follicular hyperkeratosis of discoid
•
lupus erythematosus are notably absent.
•
• Sicca syndrome if often present.
•
• Systemic symptoms are variable and can be
•
minimal (e.g., low-grade malaise, vague myalgias,
•
arthralgias) to frank impairment of another organ
•
system (e.g., renal or cardiac).
•
• About 5% to 10% of patients with SCLE have
•
vasculitis at some point during the course of their
•
disease.

16. Clinical Diagnosis
•
• The appearance of photodistributed and annular lesions is
suggestive of SCLE, particularly in a middle-aged to elderly
woman.
•
• An ANA test is positive in all cases, with anti-Ro (SSS-A)
antibodies present in more than 90% of cases.
•
• A punch biopsy (3 or 4 mm) of lesional skin that includes
subcutaneous fat often supports the diagnosis of SCLE,
although it is often impossible to exclude dermatomyositis
without clinical details.
•
• A punch biopsy (3 or 4 mm) from photodistributed nonlesional
skin for direct immunofluorescence reveals a lupus band or
other supportive findings in 60% to 80% of cases. This test is
usually only needed in problematic cases.

17. Treatment
•
• Photoprotection (e.g., hats, clothing, sun avoidance,
•
UVA sunscreen) is important.
•
• Drugs that may induce SCLE should be withdrawn.
•
• Topical corticosteroids can be tailored to the site
•
and severity of disease. In general, truncal lesions
•
require moderate to ultrapotent corticosteroids.

18. Treatment
•
• Oral prednisone (usually 20–40 mg/day) can be
•
used initially and tapered based on the response.
•
• Hydroxychloroquine (200 mg PO qd or bid) is useful,
•
but it may take 4 to 8 weeks to see improvement.
•
• Oral isotretinoin may be used as a tertiary agent, 1
•
to 2 mg/kg per day.
•
Clinical Course
•
SCLE is usually relatively easy to control, and patients
•
may go into spontaneous remission.

19. Discoid Lupus Erythematosus

20. Pathogenesis
•
Discoid lupus erythematosus (DLE), also known as
•
chronic cutaneous lupus erythematosus, is the most
•
common form of lupus to involve the skin. DLE is ninefold
•
more common in African American women than
•
in any other population, but white individuals can also
•
be affected. Like other forms of lupus erythematosus,
•
it is considered to be an autoimmune disease, yet
•
only 20% of patients with DLE have a positive ANA
•
response on laboratory testing. About 5% to 10% of
•
patients with cutaneous lesions of DLE have SLE or
•
SCLE. In contrast to other forms of lupus erythematosus,
•
drug-induced cases are uncommon.

21. Clinical Features
•
• Photodistributed lesions of DLE often affect the
•
malar face, upper chest, arms, and upper back.
•
• Primary lesions appear first as an erythematous
•
indurated plaque, often evolving to include hyperkeratosis
and dyspigmentation.
•
• On occasion, the hyperkeratosis can be considerable
•
(hypertrophic discoid lupus erythematosus).

22. Clinical Features
•
• Involvement of the conchal bowl of the ear is
•
common and distinctive (Fig. 19.24).
•
• Patients may demonstrate areas of scarring alopecia.
•
• In contrast to SLE, oral lesions are uncommon but
•
can occur occasionally.

23. Clinical Diagnosis
•
• Erythematous plaques, with hyperkeratosis,
dyspigmentation, and scarring, occurring in a
photodistribution,suggest the diagnosis of DLE.
•
• Conchal bowl involvement or a scarring alopecia are
•
clinical findings that support a diagnosis of DLE.
•
• A punch biopsy (3–6 mm) of lesional skin is strongly
•
suggestive or even diagnostic of DLE.

24. Clinical Diagnosis
•
• In select cases, a punch biopsy can also be submitted
•
for direct immunofluorescence.
•
• Recommended laboratory screening is not universally
•
agreed on but should likely include an ANA
•
test (positive in ≈20% of patients), CBC, chemistry
•
profile, and urinalysis. Some also perform complement
•
studies—C3, C4, and CH50. Laboratory
•
screening is important because 5% to 10% of
•
patients with lesions of DLE will have another form
•
of systemic lupus, SCLE or SLE.

25. Treatment
•
• Photoprotection (e.g., hats, clothing, sun avoidance,
•
UVA sunscreen) is a critical component of treatment
because the disease is driven largely by UV light.
•
• Topical corticosteroids should be tailored to the
•
site and severity of disease, but generally potent or
•
ultrapotent topical corticosteroids are used to arrest
•
the disease before large scars are formed.
•
• Intralesional triamcinolone (5–20 mg/mL) can be
•
used for select plaques.

26. Treatment
•
• Oral prednisone (starting dose, 20–40 mg/day) may
•
be used for persons with extensive disease, with a
•
taper based on the treatment response.
•
• Hydroxychloroquine (200 mg PO qd or bid) is a
•
pillar of care, but it typically takes 4 to 8 weeks
•
before significant improvement is seen. Smoking
•
can increase liver metabolism of the drug and
•
decrease its efficacy.
•
• Other medications sometimes used include chloroquine,
•
quinacrine, and thalidomide.

27. Clinical Course
•
Untreated (or incompletely treated) DLE can cause
•
permanent and disfiguring scarring and alopecia.
•
Rarely, patients with DLE without systemic disease
•
initially will progress to SLE or SCLE at a later point.
•
Although such later progression is unusual, it is useful
•
to screen persons with DLE periodically throughout
•
the course of their illness or if the course of their illness
•
seems to change in some way.