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Dermatology
Lupus Erythematosus
Built from Dermatology, 5th Edition
What’s inside
18 sections · 156 slides
Overview
- What this topic covers
History
How the disease and its subtypes were named and defined
- Naming and early observations
- Naming the cutaneous subtypes
- Diagnostic and treatment milestones
Epidemiology
Who gets lupus, and how skin-only disease compares to systemic disease
- Sex and hormonal influence
- Ethnicity and disease severity
- How common is skin-only lupus?
Pathogenesis
Genes, ultraviolet light, and the immune cascade that damages skin
- Overview: genes meet environment
- Genes that raise lupus risk
- Genes associated with systemic lupus erythematosus
- Genes associated with systemic lupus erythematosus (continued)
- Genes associated with systemic lupus erythematosus (continued)
- Genes associated with systemic lupus erythematosus (continued)
- Genes associated with systemic lupus erythematosus (continued)
- Role of anti-Ro/SSA autoantibodies
- How UV light triggers skin lupus
- Cytokines, chemokines and cell recruitment
- Type I interferon: the key amplifier
- T cells complete the attack
- UV exposure
- Pathogenesis of cutaneous lupus erythematosus
- A self-feeding inflammatory loop
Classification of cutaneous lupus
Three major forms, plus rarer chronic variants
- Gilliam's classification system
- Classification of cutaneous lupus erythematosus
- The three major forms compared
- Depth of inflammation in each cutaneous LE subtype
- Characteristic body sites of the three major forms
Discoid lupus erythematosus
The most scarring form of cutaneous lupus
- Distribution and features of discoid lesions
- Scarring, thickness, and pigment change
- Discoid lesions of lupus erythematosus, several presentations
- Discoid lupus with dyspigmentation and scarring hair loss
- Discoid lupus of the lower eyelid
- Discoid lupus in the conchal bowl of the ear
- Discoid lupus with erythema, hypopigmentation and scarring
- Scarring alopecia due to discoid lupus
- Disseminated discoid lupus lesions
- Hypertrophic (verrucous) discoid LE
- Discoid LE and the risk of systemic disease
Subacute cutaneous lupus erythematosus
A sun-triggered rash strongly linked to a specific antibody and to drugs
- Distribution and appearance of SCLE
- Two patterns of SCLE lesions
- Subacute cutaneous lupus, annular plaques on the back
- Subacute cutaneous lupus, trunk and forearm lesions
- Subacute cutaneous lupus with photodistributed plaques
- Subacute cutaneous lupus resembling erythema annulare centrifugum
- Subacute cutaneous lupus with hemorrhagic crusting
- Subacute cutaneous lupus in a V-shaped chest pattern
- Subacute cutaneous lupus on the sun-exposed arm
- Drug-induced and drug-exacerbated SCLE
- Medications associated with drug-induced SCLE
- Subacute cutaneous lupus induced by terbinafine
- Subacute cutaneous lupus possibly from a proton pump inhibitor
- SCLE, systemic disease, and anti-Ro antibodies
Acute cutaneous lupus erythematosus
The malar "butterfly rash" and its links to active systemic disease
- The malar (butterfly) rash
- Morphology and clues that point to ACLE
- Acute cutaneous lupus, variable facial erythema
- Acute cutaneous lupus involving the arms and face
- Acute cutaneous lupus sparing the nasolabial folds
- Acute cutaneous lupus of the hands
- Severe ACLE variants
- TEN-like eruption of acute cutaneous lupus
Other cutaneous lupus variants
Tumidus, panniculitis, chilblain lupus, and overlap syndromes
- Chronic cutaneous LE, the less common forms
- Lupus erythematosus tumidus
- Is LE tumidus really lupus?
- Lupus erythematosus tumidus, chest and face plaques
- Lupus erythematosus tumidus, edematous plaques
- Lupus panniculitis
- Lupus panniculitis of the upper arm
- Chilblain lupus
- Chilblain lupus of the toes
- Chilblain lupus with desquamating plaques
- Chilblain lupus genetics and Aicardi-Goutieres syndrome
- Discoid LE / lichen planus overlap
Neonatal lupus erythematosus
Maternal antibodies crossing the placenta into the baby
- Maternal anti-Ro/La
- Skin findings in neonatal lupus (NLE)
- Neonatal lupus erythematosus, forehead and scalp plaques
- Neonatal lupus, a second case of forehead and scalp plaques
- Neonatal lupus, widespread annular lesions
- Neonatal lupus, annular chest plaques
- Outcomes of the skin lesions
- Internal disease in neonatal lupus
- Systemic evaluation of an infant with cutaneous neonatal LE
- Systemic evaluation of an infant with cutaneous neonatal LE (continued)
- Counseling families after NLE
Bullous lesions of lupus
When lupus skin disease blisters
- Why blisters occur in lupus
- Bullous eruption of SLE (bullous SLE)
- Bullous eruption of systemic lupus erythematosus
Systemic lupus erythematosus
Organ involvement and how the diagnosis is classified
- SLE as a multi-organ disease
- Why classification criteria exist
- SLICC classification system: clinical and immunologic criteria
- SLICC classification system: clinical and immunologic criteria (continued)
- SLICC classification system: clinical and immunologic criteria (continued)
- SLICC classification system: clinical and immunologic criteria (continued)
- SLICC classification system: clinical and immunologic criteria (continued)
- SLICC classification system: clinical and immunologic criteria (continued)
- 2019 EULAR/ACR criteria: key design features
- 2019 EULAR/ACR classification criteria for SLE
- 1997 update of the 1982 ACR criteria for SLE classification
- 1997 update of the 1982 ACR criteria for SLE classification (continued)
- 1997 update of the 1982 ACR criteria for SLE classification (continued)
- 1997 update of the 1982 ACR criteria for SLE classification (continued)
- 1997 update of the 1982 ACR criteria for SLE classification (continued)
- 1997 update of the 1982 ACR criteria for SLE classification (continued)
- 1997 update of the 1982 ACR criteria for SLE classification (continued)
Nonspecific cutaneous lesions
Skin signs that flag systemic disease without being lupus-specific
- Vascular skin signs in lupus
- Cutaneous findings that suggest systemic lupus erythematosus
- Periungual infarcts in systemic lupus erythematosus
- Fingertip ulcerations in systemic lupus erythematosus
- Antiphospholipid-related skin findings
- Alopecia, mucin, and neutrophilic skin disease
- Nail-fold capillary changes
Pathology
What each subtype looks like under the microscope, and what antibody stains show
- Histology: shared and distinguishing features
- Characteristic histologic findings in cutaneous LE
- Characteristic histologic findings in cutaneous LE (continued)
- Histopathologic features across cutaneous LE subtypes
- Histologic features: acute versus chronic discoid LE
- CD123 staining for plasmacytoid dendritic cells
- Direct immunofluorescence (DIF)
- Direct immunofluorescence of cutaneous lupus
- When DIF is positive - and when it is not
- The lupus band test in normal-looking skin
- Clinical role of the lupus band test
Differential diagnosis and evaluation
Ruling in lupus, ruling out its many mimics
- Differential diagnosis varies by subtype
- Differential diagnoses of cutaneous lupus subtypes
- Approaching the diagnosis
- Diagnostic algorithm for cutaneous lupus
- Evaluating for systemic disease
- Evaluation for systemic lupus erythematosus
- Interpreting the ANA test
- Drug-induced lupus: two different pictures
- Drug-induced SCLE versus drug-induced SLE
- Limits of classification criteria
Treatment
Topical care, antimalarials, and options for refractory disease
- Topical and intralesional therapy
- Therapy of cutaneous lupus erythematosus
- Therapy of cutaneous lupus erythematosus (continued)
- Antimalarial therapy: the systemic gold standard
- Antimalarial safety: eye toxicity
- Options for antimalarial-refractory disease
- B-cell and biologic therapies
- Mechanisms of newer monoclonal antibody therapies
- Topical/intralesional steroids
- Systemic therapy for organ-threatening SLE
- Sun protection: a core part of therapy
- Other adjunctive measures
Summary
The essentials to carry forward
- Key takeaways
References
Source list from the chapter
- References
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- References (continued)
- Dermatology, 5th Edition (2-Volume Set)