Cutaneous manifestatio of SLE: Diagnosis and Management

1. CUTANEOUS
MANIFESTATION OF SLE:
DIAGNOSIS AND
MANAGEMENT
DR. SETH KYEI – FRAM

2. INTRODUCTION
• Lupus erythematosus (LE) is a complex autoimmune disease with heterogeneous
cutaneous and systemic manifestations that can evolve over the course of disease.
• It is characterized by antibodies to nuclear and cytoplasmic antigens
• Multisystemic inflammation with variable clinical manifestations
• Has a predilection for women of childbearing age
• In 2019 the American College of Rheumatology (ACR) and the European League
Against Rheumatism (EULAR) published new criteria for the classification of SLE.

4. Manifestation of SLE
• Patients may present with any of the following;
• Constitutional (eg, fatigue, fever, arthralgia, weight changes)
• Musculoskeletal (eg, arthralgia, arthropathy, myalgia, frank arthritis)
• Dermatologic (eg, malar rash, photosensitivity)
• Renal (eg, acute or chronic kidney failure, acute nephritic disease)
• Neuropsychiatric (eg, seizure, psychosis)
• Pulmonary (eg, pleurisy, pleural effusion, pneumonitis, pulmonary hypertension,
interstitial lung disease)
• Gastrointestinal (eg, nausea, dyspepsia, abdominal pain)
• Cardiac (eg, pericarditis, myocarditis)
• Hematologic (eg, leukopenia, lymphopenia, anaemia, or thrombocytopenia)

5. Manifestation of SLE
• Patients may present with any of the following;
• Constitutional (eg, fatigue, fever, arthralgia, weight changes)
• Musculoskeletal (eg, arthralgia, arthropathy, myalgia, frank arthritis)
• Dermatologic (eg, malar rash, photosensitivity)
• Renal (eg, acute or chronic kidney failure, acute nephritic disease)
• Neuropsychiatric (eg, seizure, psychosis)
• Pulmonary (eg, pleurisy, pleural effusion, pneumonitis, pulmonary hypertension,
interstitial lung disease)
• Gastrointestinal (eg, nausea, dyspepsia, abdominal pain)
• Cardiac (eg, pericarditis, myocarditis)
• Hematologic (eg, leukopenia, lymphopenia, anemia, or thrombocytopenia)

6. Cutaneous Lupus Erythematosus (CLE)
• CLE can occur as a manifestation of SLE or independent of SLE.
• CLE is classified into:
• LE-specific skin lesions
• The key characteristic that unites the LE-specific skin diseases is histopathology
• Vacuolar interface dermatitis; hyperkeratosis; epidermal atrophy; a superficial, perivascular, and
perifollicular mononuclear cell inflammatory infiltrate; thickening of the basement membrane; and
pigment incontinence.
• LE-nonspecific skin lesions
• LE-nonspecific lesions lack histologic features of LE, but occur with increased frequency in patients
with SLE.

10. LE-specific skin lesions
• In most patients, one form of LE-specific skin involvement will predominate.
• However, overlapping features may occur.
• The lesions are distinguished primarily by the location of the inflammatory infiltrate and
not by the chronicity of the lesions.

11. Acute cutaneous lupus erythematosus (ACLE)
• ACLE is the most common form of cutaneous lesions of lupus
associated with SLE.
• Occurs in 30% to 50% of patients with SLE.
• Systemic involvement is typical and rashes often flare in
parallel with other organ disease activity
• Ninety-five percent of patients with ACLE have positive ANA

12. Acute cutaneous lupus erythematosus (ACLE)
• UV exposure is a common exogenous factor capable of
precipitating ACLE
• A possible association with HLA-DR2 and -DR3 has been
suspected
• May present as
• Localized ACLE (ie, malar rash, butterfly rash)
• Generalized ACLE
• Toxic epidermal necrolysis-like ACLE

13. 1. Localized ACLE
• This is the typical manifestation of ACLE
• It is characterized by a localized erythema known as the “malar rash” or
“butterfly rash” on the central portion of the face.
• The nasolabial folds are spared.
• Localized ACLE may precede other symptoms of SLE by months or
even years or may be accompanied by other symptoms and signs of
acute SLE.
• May only affect the skin transiently, and the lesions may last for only
several days up to a few weeks

14. 1. Localized ACLE
• Usually begins with small, discrete erythematous macules and
papules
• Often recurs, particularly with sun exposure
• Lesions may develop scales
• It can disappear without scarring and although
dyspigmentation can occur

18. Differential diagnosis
• Rosacea
• involvement of the nasolabial fold, papules or pustules
• worsens with specific triggers such as alcohol, heat, and spicy foods
• Dermatomyositis
• involves the nasolabial folds
• Sunburn
• Seborrheic dermatitis
• Contact dermatitis
• Flushing (carcinoid)

19. (A) The malar rash of ACLE refers to erythema over the nasal bridge and cheeks that spares the nasolabial folds. (B) Facial erythema in
dermatomyositis tends to involve the nasolabial folds. (C) Rosacea can mimic the facial erythema of ACLE but tends to worsen with specific
triggers such as alcohol, heat, and spicy foods.

20. Severe erythema,
papules, and
pustules in Rosacea

21. A. Butterfly rash in
systemic lupus
erythematosus. A well-
demarcated, symmetrical
erythema of the malar
areas and the back of the
nose that has progressed
to the forehead and
perioral skin. Note the
sparing of the nasolabial
folds.
B. Seborrheic dermatitis:
note the yellowish colour
and involvement of the
nasolabial folds

22. 2. Generalized ACLE
• Also known as “photosensitive lupus rash”
• A less common variety of ACLE and may be located anywhere on the body
• It has a predilection for sun-exposed areas of the face, extensor aspects of the
arms and forearms, and the dorsal aspects of the hands.
• It presents often as pruritic, widespread eruption of symmetric macules and
papules that is photosensitive.
• Notably, the skin overlying the knuckles often is spared

24. Differential diagnosis
• Dermatomyositis
• Involvement of the metacarpophalangeal and interphalangeal joints
•
Dermatomyositis of the hands often shows confluent erythema of the
skin overlying the MCP and IP joints and the extensor tendons

25. 3. Toxic epidermal necrolysis-like ACLE
• Occasionally, the inflammatory infiltrate is severe enough to produce vesicles or
bullae resembling toxic epidermal necrolysis.

26. • Nonscarring, non–atrophy-producing, photosensitive dermatosis
• Commonly involved sites are sides of face, V of the neck, extensor forearms
• Midfacial skin is usually spared
• SCLE is believed to occur in 10% to 15% of patients with SLE
• It may be drug induced
• HCT; ACE-I; CCB, terbinafine, NSAIDs, PPIs, AEDs and chemotherapy agents
• Lesions may/not clear once the medication is discontinued
• Also occurs in patients with Sjögren syndrome
• A strong association exists with anti-Ro (SS-A) autoantibodies
Subacute cutaneous lupus erythematosus-SCLE

27. • Several LE-nonspecific lesions have been described in patients with SCLE
• nonscarring alopecia, painless mucous membrane lesions, livedo reticularis,
periungual telangiectasias, and Raynaud’s phenomenon
• Patients with SCLE may also develop localized facial ACLE
Subacute cutaneous lupus erythematosus-SCLE

28. • Begins with erythematous macules and papules that evolve into scaly
papulosquamous or annular/polycyclic plaques
• Lesions usually resolve without scarring, though dyspigmentation
may occur
• permanent vitiligo-like pigmentary changes
• Subtypes of SCLE include
• Papulosquamous SCLE
• Annular SCLE
• Some patients exhibit features of both subtypes
Subacute cutaneous lupus erythematosus-SCLE

29. Hypopigmentation in subacute cutaneous
lupus erythematosus (SCLE). Permanent
vitiligo-like depigmentation in the face of a
patient with SCLE

30. • The primary lesion is an erythematous papule or a small
plaque, often with slight scaling.
• Lesions expand and may merge and eventually form plaques
with scaling
• The papulosquamous variant can
resemble eczema or psoriasis, as well as pityriasis in some
instances
1. Papulosquamous SCLE

31. Papulosquamous SCLE
Papulosquamous SCLE. Psoriasiform
lesions with superficial scale and the tendency
for individual lesions to merge into a vetiform
pattern

32. Papulosquamous lesions of subacute cutaneous lupus erythematosus may
simulate psoriasis.
Papulosquamous SCLE

33. SCLE, papulosquamous.
Psoriaform lesions which
coalesce to form retiform
arrays
Papulosquamous SCLE

34. 2. Annular SCLE
• Presents with scaly annular erythematous
plaques, which often merge to form a polycyclic
morphology
Polycyclic lesions with central hypopigmentation and
inflamed erythematous borders on the extensor aspects of
the arm

35. Annular SCLE
SCLE, annular polycyclic. Scaly annular
erythematous plaques coalesce into polycyclic
arrays.

36. Annular SCLE
Annular SCLE lesions seen on the
arm and chest

37. Chronic cutaneous lupus erythematosus
(CCLE)
• CCLE is notable for demonstrating a chronic, recurrent course which typically
requires long-term treatment
• CCLE has several subtypes, including
• Discoid lupus erythematosus (DLE)
• Lupus erythematosus tumidus (LE tumidus)
• Lupus profundus (lupus panniculitis)
• Chilblain lupus erythematosus (chilblain LE)

38. Discoid lupus erythematosus (DLE)
• DLE is the most common subtype of CCLE, representing 50% of cases
• Localized if it involves exclusively the head and neck area
• Generalized if it extends below the neck with a predilection for the upper extremity extensor
surfaces
• DLE begins with a flat or slightly elevated, sharply demarcated, erythematous
macules or papules with a scaly surface.
• Early lesions evolve into larger, coin-shaped (“discoid”), confluent, disfiguring
plaques of varying size
• Resolution of the lesions leaves evident atrophy and scarring

39. Discoid lupus erythematosus (DLE)
• Involvement of the scalp can be found in approximately 60% of patients and may
result in in irreversible scarring alopecia
• Mucous membrane involvement can be found in 25% of patients with DLE
• May affect buccal, palate, alveolar processes, tongue, nasal, conjunctival, anogenital
mucous membranes
• Infrequently, squamous cell carcinoma develops in sites of DLE (2-3%)

40. Discoid lupus
erythematosus (DLE)
Discoid lupus erythematosus:
disfiguring, lesion showing erythema
and peripheral hyperchromia.

41. Discoid lupus
erythematosus (DLE)
Classic discoid lupus erythematosus
(DLE). Slightly infiltrated, erythematous
plaques with scarring atrophy in a patient
with therapeutically refractory facial DLE

42. Discoid lupus
erythematosus (DLE)
Perioral pitted scarring in discoid lupus
erythematosus (DLE).
Perioral DLE lesions often resolve with
a striking acneiform pattern of pitted
scarring

43. Discoid lupus erythematosus (DLE)
Discoid lupus erythematosus (DLE) of the scalp. Irreversible scarring alopecia as a
result of persistent activity in localized areas

44. Discoid lupus erythematosus (DLE)
DLE of the scalp. Longstanding discoid lesions show
atrophy, with hyperpigmentation peripherally and
depigmentation centrally.

45. Discoid lupus
erythematosus (DLE)
Mucosal discoid
lupus erythematosus (DLE).
Typical appearance of a buccal
lesion on the hard palate with a
honeycomb appearance

46. Discoid lupus
erythematosus (DLE)
Cheilitis in discoid
lupus erythematosus (DLE).
Diffuse small lesions on the vermillion
border of the upper lip
causing considerable discomfort

47. Lupus erythematosus tumidus (LET)
• LET lesions tend to occur on the face, neck, upper chest, and shoulders
• The lesions spare the knuckles, inner aspect of the arms, and axillae
• Consist of erythematous macules, papules, and plaques, normally with smooth
surfaces
• Scarring, the hallmark of DLE, does not occur in LET

48. Lupus erythematosus
tumidus (LET)
Lupus erythematosus tumidus (LET).
Succulent, elevated, urticaria-like erythematous
plaques on the right cheek

49. Lupus erythematosus
tumidus (LET)
Polycyclic/annular form of lupus
erythematosus tumidus (LET). Confluent,
nonscarring lesions on the face with
a tendency to coalesce in the
periphery and flatten in the center

50. • A rare variant of CCLE in which pathologic changes occur primarily in the lower
dermis and subcutaneous tissue
• Lupus profundus presents as indurated plaques or nodules with or without overlying
cutaneous changes
• The plaques or nodules may appear on the scalp, face, upper arms, chest (particularly
breasts), lower back, flank, upper thighs, or buttocks
• Upon resolution, lupus profundus may leave depressed areas of lipoatrophy
• The major morbidity is usually disfigurement and disability related to pain
• LEP may produce breast nodules that can mimic carcinoma, clinically and
radiologically
Lupus Erythematosus profundus (lupus panniculitis)

51. Lupus Erythematosus
profundus (lupus panniculitis)
Lupus erythematosus profundus
(LEP). Subcutaneous nodules leaving extensive
depressed, atrophic areas on the upper arm with
hyperpigmented borders

52. Lupus Erythematosus
profundus (lupus panniculitis)

53. • The term "chilblains" is derived from two Old English words "chill" (cold) and
"blegen" (sore)
• Presents with tender, bright red to reddish-blue papules, nodules, or plaques on the
toes, fingers, nose, or ears precipitated by cold exposure
• The risk of developing SLE is estimated to be approximately 20%
• The lesions of CHLE involve mostly the dorsal and lateral parts of the hands and
feet, the ears, the nose, the elbows, the knees, or the calves
Chilblain lupus erythematosus (chilblain LE)

54. Chilblain lupus erythematosus (chilblain LE)
Chilblain lupus erythematosus
(CHLE). Red-purple patches on the finger end
joints that are precipitated by cold, damp
climates

55. Diagnosis
• Mainly clinical
• Supported by contextual clinical features (such as the presence of known underlying
SLE)
• Confirmatory histopathologic examination is indicated when diagnostic uncertainty
remains.

56. Management
• The goal in the management prevent and treat skin activity to minimize damage.
• Modalities of treatment include:
• Prevention
• Topical or intralesional corticosteroids, topical calcineurin inhibitors, and/or systemic
glucocorticoids
• Systemic antimalarial agents (hydroxychloroquine or chloroquine)
• Methotrexate
• Mycophenolate mofetil
• Thalidomide, lenalidomide, belimumab, dapsone, IVIG, azathioprine

57. Prevention
• Photoprotection
• application of a broad-spectrum sunscreen
• Protective clothing
• Avoiding exposure during peak sunlight hours
• Smoking cessation
• Vitamin D supplementation

58. Topical steroids
• Topical corticosteroids are the mainstay in the treatment of localized CLE
• Topical steroids should be applied time-limited (2-4 weeks) and preferably intermittent
• Side effects: atrophy, telangiectasias, steroid dermatitis, and folliculitis
• To minimize the side effects:
• Twice-daily application for a few weeks
• Followed by a rest period of a few weeks

59. Topical Calcineurin Inhibitors
• Topical calcineurin inhibitors (0.03% and 0.1% tacrolimus ointment, 1% pimecrolimus
cream)
• The major advantage of these agents is their better safety profile if compared with
topical corticosteroids
• Can be used as alternative first-line or as a second-line topical treatment option.

60. Antimalarials
• Antimalarials include hydroxychloroquine, chloroquine, and quinacrine.
• They are considered the first-line systemic treatment in all subtypes of CLE
• The main side effect of HCQ and CQ is retinal toxicity
• Ophthalmological consultation
• Screen for G6PD deficiency
• If monotherapy with HCQ or CQ is not successful, quinacrine (100 mg/day) may be
added
• The most frequent side effect of quinacrine is yellow discoloration of the skin and
mucous membranes.
• Rarely aplastic anaemia may develop

61. Other agents
• Methotrexate (MTX)
• Additional benefits may include treatment of SLE with inflammatory arthritis component.
• Retinoids
• Isotretinoin; Acitretin
• Dapsone
• Effective in bullous lupus erythematosus (maybe used as first line)
• Mycophenolate Mofetil (MMF)
• Dual benefit to patients with underlying lupus nephritis, interstitial lung disease.
• Azathioprine, cyclophosphamide, and cyclosporine
• Not recommended for CLE patients without systemic organ involvement.

63. Summary
• The spectrum of cutaneous disease in SLE is extremely broad and can
occur at any point in the disease
• Diagnosis of CLE is mainly clinical
• Histopathological examination can be performed when diagnosis is in
doubt.
• Timely and appropriate therapy to control activity and minimize damage
is the goal of treatment.

64. Reference
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