Cutaneous Lupus Erythematosus

There are more patients with cutaneous LE without systemic lupus erythematosus (SLE) than patients with SLE according to population-based studies

True

Which of the following is NOT considered an environmental factor in the pathogenesis of cutaneous LE?

a) Ultraviolet radiation (UVR)

b) Medications

c) Cigarette smoking

d) Genetic predisposition

Genetic predisposition

lichenoid tissue reaction is a feature of most subsets of cutaneous LE?

True

Who first used the term 'lupus erythematosus'?

Cazenave

Which of the following is NOT a major form of cutaneous LE according to the Gilliam classification schema?

a) Acute cutaneous LE (ACLE)

b) Subacute cutaneous LE (SCLE)

c) Chronic cutaneous LE (CCLE)

d) Intermittent cutaneous LE

Intermittent cutaneous LE

patients with discoid LE (DLE) lesions have a higher likelihood of progressing to SLE

False

What is a typical characteristic of SCLE lesions?

Lesions may have an annular configuration with raised borders and central clearing.

the malar rash of ACLE typically spares the nasolabial folds?

True

Which autoantibody is NOT specifically associated with SLE?

a) Anti-dsDNA

b) Anti-Sm

c) Anti-SSA/Ro

d) Anti-RNP

Anti-RNP

What is a characteristic histologic finding of discoid LE lesions?

a) Prominent dermal mucin deposition

b) Periadnexal inflammation and follicular plugging

c) Subcutaneous fat inflammation

d) Superficial lymphocytic infiltrate.

Periadnexal inflammation and follicular plugging.

a positive ANA test (titer ≥1:80) is an entry criterion according to the 2019 EULAR/ACR classification criteria for SLE

True

How many points are given for subacute or discoid lupus if observed by a clinician according to the 2019 EULAR/ACR classification?

a) 2 points

b) 3 points

c) 4 points

d) 6 points

4 points

Drug-induced SLE commonly presents with nephritis and CNS disease

False

According to the SLICC classification system for SLE, how many of 17 criteria must be met for a diagnosis?

a) 2

b) 3

c) 4

d) 5

4

What is a typical feature of ACLE?

a) Chronic, relapsing course.

b) Annular configuration with raised borders.

c) "Butterfly rash"

d) Significant scarring

'Butterfly rash'.

Which condition is typically associated with anti-SSA/Ro autoantibodies? ◦

a) Acute cutaneous LE (ACLE)

b) Discoid LE (DLE)

c) Lupus panniculitis (LEP)

d) Subacute cutaneous LE (SCLE)

Subacute cutaneous LE (SCLE)

Patients with SLE have more prominent nail-fold capillary abnormalities than patients with systemic sclerosis

True

Which of the following is NOT a typical non-specific cutaneous finding in LE?

a) Raynaud phenomenon

b) Livedo reticularis

c) Palmar erythema

d) Gottron's papules

Gottron's papules

What is the primary location of inflammatory infiltrates in LE tumidus?

a) Superficial dermis

b) Superficial and deep dermis perivascular and periadnexal

c) Subcutaneous fat

d) Epidermis

Superficial and deep dermis perivascular and periadnexal.

In the past, "ANA-negative SLE" was more common, especially in patients with anti-SSA/Ro antibodies, but is now rare due to the use of human HEp-2 cells in ANA assays

True

Anti-histone antibodies are a serologic marker of drug-induced ANA and drug-induced SLE, and can also be present in patients with idiopathic SLE

True

Phototherapy protocols such as PUVA or NB-UVB significantly induce the appearance of ANA and other autoantibodies

False

Which autoantibodies are included in the current classification criteria for systemic sclerosis?

a) Anti-centromere (CENP-B); anti-topoisomerase I; and anti-RNA polymerases.

b) Anti-Mi-2; Anti-TIF1-γ; Anti-MDA5

c) Anti-Jo-1; Anti-topoisomerase-1; Anti-RNA polymerase III

d) Anti-fibrillin-1; anti-histones; anti-ssDNA

Anti-centromere (CENP-B); anti-topoisomerase I; and anti-RNA polymerases.

Which of the following is NOT a clinical feature of mixed connective tissue disease (MCTD)?

a) High-titer IgG anti-U1 ribonuclear protein (U1RNP) antibodies

b) Raynaud phenomenon

c) Gottron's papules

d) Esophageal dysmotility

Gottron's papules.

Essay Q1: ◦

Describe the typical morphology and distribution of ACLE lesions, and list three features that help distinguish it from other facial eruptions.

-

Essay Q2: Explain the association between ACLE and systemic disease, including the autoantibodies and internal organ involvement that are commonly seen.

-

Essay Q3: Discuss the pathogenesis of ACLE, including the inflammatory pathways and cells involved, according to the source

Essay Q4: Compare and contrast the clinical and histological features of ACLE with SCLE and DLE, using information from the text and figures provided in the sources.

-

What is the significance of the "butterfly rash" in the diagnosis of ACLE?

-

What is the female-to-male ratio of systemic lupus erythematosus (SLE) patients during childbearing years?

Women outnumber men by at least 9:1.

In terms of ethnicity, which group has a higher prevalence of SLE in the US?

African-American women, with a prevalence of 211 per 100,000 compared to 64 per 100,000 for White women.

What are common environmental triggers for cutaneous lupus erythematosus (LE)?

Ultraviolet radiation (UVR), medications, cigarette smoking, and possibly viruses.

What type of skin reaction is commonly associated with cutaneous lupus erythematosus?

A lichenoid tissue reaction, characterized by epidermal basal cell damage and lymphocytic infiltrate in the dermis.

What are the variants of cutaneous lupus erythematosus?

Acute Cutaneous LE (ACLE), Subacute Cutaneous LE (SCLE), and Chronic Cutaneous LE (CCLE).

Describe the lesions associated with Discoid Lupus Erythematosus (DLE).

Lesions are long-lived, intensely inflammatory, and can lead to permanent scarring with features like follicular plugging.

What is Lupus Erythematosus Tumidus (LET) characterized by?

Firm, erythematous plaques that lack scale or follicular plugging.

What diagnostic criteria does the SLICC classification system require?

At least 4 of 17 criteria including at least one clinical and one immunologic criterion.

What laboratory tests are recommended for cutaneous lupus?

ANA with profile, urinalysis, CBC, chemistries, ESR, CRP, complement levels, and antiphospholipid antibodies.

What is a common treatment for cutaneous lupus erythematosus?

General measures include sunscreen, avoiding photosensitizing medications, and possible use of topical corticosteroids.

What are some potential complications of cutaneous lupus erythematosus?

Scarring, disfigurement, dyspigmentation, scarring alopecia, and progression to systemic lupus erythematosus (SLE).

Which autoantibodies are relatively specific for SLE?

Autoantibodies to dsDNA and Sm.

What are the common differential diagnoses for Acute Cutaneous LE?

Rosacea, seborrheic dermatitis, sunburn, drug-induced photosensitivity, and dermatomyositis.

What is Acute Cutaneous Lupus Erythematosus (ACLE)?

ACLE is a subtype of cutaneous lupus erythematosus characterized by transient lesions, often after sun exposure.

What is a typical presentation of ACLE?

Malar erythema, commonly known as the 'butterfly rash', is a typical presentation.

How do ACLE lesions typically resolve?

The lesions usually resolve without scarring but may cause dyspigmentation.

What systemic condition is ACLE often associated with?

ACLE is often associated with active systemic lupus erythematosus (SLE).

What are common oral symptoms of ACLE?

Oral ulcerations are not uncommon in ACLE.

What environmental factors contribute to the pathogenesis of ACLE?

Environmental factors include ultraviolet radiation (UVR), medications, cigarette smoking, and possibly viruses.

What immune response is activated by UVR in the context of ACLE?

UVR induces apoptosis and increases secretion of proinflammatory cytokines and interferons.

What are the characteristic histopathological findings in ACLE?

Mild interface dermatitis with vacuolization of basal keratinocytes and sparse superficial lymphoid infiltrates.

What kind of therapy is considered a gold standard for systemic treatment of ACLE?

Antimalarials, such as hydroxychloroquine, are considered the gold standard.

What is Rowell syndrome in the context of lupus?

Rowell syndrome is characterized by erythema multiforme-like lesions that may occur in lupus patients.

What is a major complication of ACLE?

Dyspigmentation and progression to systemic lupus erythematosus (SLE) are major complications.

What key laboratory tests are used to evaluate ACLE?

Key lab tests include ANA with profile (anti-dsDNA, -Sm), urinalysis, CBC, chemistries, ESR, CRP, and complement levels.

What differentiates malar erythema of ACLE from other facial eruptions?

The presence of telangiectasias, erosions, dyspigmentation, and epidermal atrophy help to distinguish malar erythema of ACLE.