Chilblain Lupus Erythematosus Overview
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Questions and Answers

What is one method recommended for evaluating quality of life in patients with cutaneous lupus erythematosus (CLE)?

  • ECLAM
  • SELENA-SLEDAI
  • DLQI or Skindex-29 (correct)
  • ECLAM and BILAG

    • Which activity score is focused on systemic lupus erythematosus but is not ideal for assessing cutaneous symptoms?

  • ECLAM
  • CLASI/RCLASI
  • BILAG (correct)
  • Skindex-29

    • Which of the following statements is true regarding the reimbursement of pacrine?

  • It is frequently not reimbursed. (correct)
  • It is fully covered by health insurance.
  • It is only used for cosmetic treatments.
  • It is always mentioned as a first-line treatment.

    • What factor should be considered during treatment monitoring related to quality of life?

    <p>Cosmetic aspects</p> Signup and view all the answers

    Which score is specifically indicated for assessing disease activity in SLE?

    <p>BILAG</p> Signup and view all the answers

    What is the primary purpose of the DLQI and Skindex-29?

    <p>To measure quality of life related to skin conditions</p> Signup and view all the answers

    Why might traditional activity scores not be suitable for assessing cutaneous lupus erythematosus (CLE)?

    <p>They do not address skin involvement adequately.</p> Signup and view all the answers

    What is a common limitation of the pacrine treatment mentioned?

    <p>Frequent reimbursement issues</p> Signup and view all the answers

    What is the classic screening test for connective tissue diseases?

    <p>Antinuclear antibodies (ANA) determination</p> Signup and view all the answers

    Which antibodies are typical for subacute cutaneous lupus erythematosus (SCLE)?

    <p>Anti-Ro/SS-A antibodies</p> Signup and view all the answers

    What do low levels of complement C3 and C4 indicate in the context of lupus?

    <p>Monitoring disease courses and activity in SLE</p> Signup and view all the answers

    Which antibodies are frequently found in drug-induced lupus erythematosus?

    <p>Anti-histone antibodies</p> Signup and view all the answers

    What is the role of antinuclear antibodies (ANA) in the diagnosis of systemic lupus erythematosus (SLE)?

    <p>ANA is an obligatory criterion for diagnosis</p> Signup and view all the answers

    What indicates that antiphospholipid antibodies are more indicative of SLE rather than CLE?

    <p>Detection of significant APS-AB levels</p> Signup and view all the answers

    What is the frequency range for antiphospholipid antibodies found in various CLE subtypes?

    <p>5.8% to 68%</p> Signup and view all the answers

    Which of the following antibodies is NOT typical for cutaneous lupus erythematosus (CLE)?

    <p>Anti-ds-DNA antibodies</p> Signup and view all the answers

    What characterizes interface dermatitis as seen in chilblain lupus erythematosus (CHLE)?

    <p>Infiltration of the basal epidermal layer with cytotoxic lymphocytes and plasmacytoid dendritic cells</p> Signup and view all the answers

    Which of the following locations is NOT a common predilection site for chilblain lupus erythematosus?

    <p>Forehead</p> Signup and view all the answers

    What triggers the inflammatory response in chilblain lupus erythematosus?

    <p>Drop in temperature and cold exposure</p> Signup and view all the answers

    Which immune responses are involved in chilblain lupus erythematosus?

    <p>Both adaptive and innate immune responses</p> Signup and view all the answers

    What is a significant pro-inflammatory factor associated with chilblain lupus erythematosus?

    <p>Type I/III interferons and associated cytokines like CXCL10</p> Signup and view all the answers

    Which symptom is characteristically associated with chilblain lupus erythematosus?

    <p>Painful livid swellings and ulceration</p> Signup and view all the answers

    The clinical and histological differentiation from genuine chilblains is considered to be:

    <p>Difficult due to overlapping characteristics</p> Signup and view all the answers

    What type of immune cells are primarily involved in the basal layer during chilblain lupus erythematosus?

    <p>Cytotoxic lymphocytes and plasmacytoid dendritic cells</p> Signup and view all the answers

    What is the primary focus of the classification by Kuhn and Ruzicka in 2004?

    <p>Classification of cutaneous lupus erythematosus</p> Signup and view all the answers

    Which publication discussed the epidemiology and socioeconomic impact of skin disease in lupus erythematosus?

    <p>Lupus 1997</p> Signup and view all the answers

    What specific subgroup of cutaneous lupus erythematosus does Kuhn, Ruzicka, and others report as neglected?

    <p>Lupus erythematosus tumidus</p> Signup and view all the answers

    Which autoantigen's expression is upregulated in patients with dermatomyositis according to Scholtissek and colleagues?

    <p>TRIM33/TIF1gamma</p> Signup and view all the answers

    What is a primary research focus of the work published by Jimenez et al. in relation to lupus erythematosus?

    <p>Epidemiology of cutaneous lupus erythematosus</p> Signup and view all the answers

    In which year was the study by Kuhn and Landmann regarding the classification and diagnosis of cutaneous lupus erythematosus published?

    <p>2004</p> Signup and view all the answers

    What type of manifestations are primarily discussed in the work of Sontheimer and Provost related to lupus erythematosus?

    <p>Cutaneous manifestations</p> Signup and view all the answers

    What characterizes scarring skin lesions of discoid lupus erythematosus?

    <p>Strong expression of the type I interferon-induced protein MxA</p> Signup and view all the answers

    Which journal published the study on the expression of TRIM33/TIF1gamma and its association with cellular stress markers?

    <p>Clin Exp Dermatol</p> Signup and view all the answers

    Which type of cells are emphasized in the presence and distribution in various inflammatory skin diseases?

    <p>Plasmacytoid dendritic cells</p> Signup and view all the answers

    What is the implication of ultraviolet A exposure for photosensitive patients?

    <p>Potential exacerbation of symptoms</p> Signup and view all the answers

    Which study discusses the composition of the lupus band test on sun-protected non-lesional skin?

    <p>Cardinali et al. 1999</p> Signup and view all the answers

    What type of sun protection strategies are discussed for patients with lupus erythematosus?

    <p>Broad-spectrum photoprotection</p> Signup and view all the answers

    What is not typically associated with the diagnosis and treatment of photosensitivity in lupus patients?

    <p>Topical steroid treatments</p> Signup and view all the answers

    Which of the following studies evaluated the capacity of sunscreens to photoprotect lupus erythematosus patients?

    <p>Faurschou et al. 2007</p> Signup and view all the answers

    What factor is related to the effectiveness of sun protection in lupus erythematosus patients, according to the studies?

    <p>Amount of sunscreen applied</p> Signup and view all the answers

    Study Notes

    Cutaneous Lupus Erythematosus (CLE)

    • ACR criteria from 1982 are fulfilled in 35–50% of cases.
    • Interface dermatitis is a histological manifestation linked to anti-epidermal inflammation in lupus.

    Chilblain Lupus Erythematosus (CHLE)

    • Characterized by painful, livid swellings and large, cushion-like nodes that may erode or ulcerate.
    • Predominantly found in symmetrical acral areas exposed to cold (e.g., fingers, toes, ears).
    • Symptoms often worsen in cold and damp conditions, particularly during winter.

    Immune Response Mechanisms

    • Involves both adaptive (autoantibodies, T cells) and innate immune responses (cell death, cytokine pathways).
    • Key pro-inflammatory factors include type I/III interferons and cytokines like CXCL10, produced by plasmacytoid dendritic cells and keratinocytes.

    Diagnosis

    • Antinuclear antibody (ANA) tests are essential for diagnosing connective tissue diseases, particularly SLE, with low titers (≤ 1:320) often seen in CLE.
    • Positive ANA is required for SLE diagnosis; further testing for ENA is recommended to specify subtypes.

    Antibodies Associated with CLE

    • Anti-Ro/SS-A antibodies are common in subacute cutaneous lupus erythematosus (SCLE).
    • Anti-histone antibodies are frequently present in drug-induced lupus erythematosus, while anti-ds-DNA and anti-Sm antibodies align with SLE.

    Antiphospholipid Syndrome (APS)

    • Antiphospholipid antibodies (APS-AB) such as cardiolipin and lupus anticoagulant are part of the SLE diagnostic criteria and vary in frequency among CLE types (5.8–68%).
    • Low complement levels (C3 and/or C4) are typical in SLE, used to monitor disease progression, unlike normal levels in CLE.

    Quality of Life Assessments

    • Quality of life in CLE patients can be evaluated using dermatological indices like DLQI (Dermatology Life Quality Index) and Skindex-29, especially relevant due to scarring effects.
    • Activity scores like ECLAM, BILAG, SELENA-SLEDAI, and SLAM assess SLE but lack specificity for cutaneous manifestations.

    Treatment Considerations

    • Treatments may be influenced by cosmetic concerns related to scarring in facial and scalp lesions.
    • The use of general dermatological quality of life scores is recommended for tracking treatment efficacy and patient satisfaction.

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    Description

    This quiz covers the histological and clinical aspects of Chilblain lupus erythematosus (CHLE) and its association with systemic lupus erythematosus (SLE). Test your knowledge on the ACR criteria from 1982 and the characteristics of interface dermatitis. Understand the clinical appearance and underlying mechanisms of this condition.

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