791 results match your criteria: "Linear IgA Dermatosis"

Linear IgA bullous dermatosis: 32 years of experience.

An Bras Dermatol

December 2024

Department of Dermatology and Venereology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.

Background: Linear IgA bullous dermatosis (LABD) is an uncommon disease with only a few reported studies in large series with long follow-up periods.

Objectives: To evaluate the clinical presentation, immunopathological features, management, and disease course in LABD patients.

Methods: Data including demographics, clinical features, histopathological and immunofluorescence findings of LABD patients, in addition to the preferred treatments and responses to treatments were evaluated.

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Article Synopsis
  • - Dermatitis herpetiformis (DH) and linear IgA bullous dermatosis (LABD) are skin conditions identifiable by specific patterns of IgA deposition observed through direct immunofluorescence (DIF) testing, which may include other immunoglobulin deposits. - An analysis comparing 45 LABD cases to 48 DH cases revealed that the "picket fence pattern" was absent in LABD but present in nearly half of the DH cases, along with differences in the levels of IgG, IgM, and kappa light chains between the two conditions. - The "picket fence pattern" is a highly specific (100%) but not very sensitive (47.9%) indicator for diagnosing DH, potentially aiding clinicians
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Article Synopsis
  • Linear IgA bullous dermatosis (LABD) is a rare autoimmune condition typically treated with dapsone, though other immunomodulators are used if needed.
  • A recent case involved a 12-year-old boy with a severe skin condition diagnosed as LABD, confirmed through biopsy, and treated effectively with prednisone and cyclosporine.
  • The patient's condition improved significantly within 2 weeks, leading to further maintenance therapy with cyclosporine after initial treatments were paused due to side effects.
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Uncommon and Unusual Variants of Autoimmune Bullous Diseases.

Indian Dermatol Online J

August 2024

Department of Dermatology, Venereology and Leprology, Kasturba Medical College Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India.

Article Synopsis
  • Autoimmune blistering diseases (AIBDs) are skin conditions caused by the body's immune system attacking certain proteins, leading to blister formation, and can be categorized into intraepidermal and subepidermal types.
  • The objective of this review is to raise awareness about rare and uncommon AIBD variants, such as pemphigus herpetiformis and anti-laminin 332 pemphigoid, among others.
  • By discussing these unusual variants, the review aims to assist healthcare professionals in the early diagnosis and treatment of these conditions.
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Linear immunoglobulin A bullous dermatosis induced by atezolizumab.

Dermatol Reports

September 2024

Division of Dermatology, King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Riyadh.

Article Synopsis
  • Linear immunoglobulin A bullous dermatosis (LABD) is a rare skin condition that causes blisters and can be triggered by infections or medications.
  • Atezolizumab, a monoclonal antibody used for cancer treatment, affects a specific immune pathway.
  • The text presents a case where LABD occurred as a side effect after a patient received Atezolizumab.
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Article Synopsis
  • * This study analyzed the histopathologic characteristics of 11 cases of each disease, noting that both conditions commonly present with itchy blisters and certain similar microscopic findings, but with differences in eosinophil presence and specific epidermal changes.
  • * Ultimately, the researchers concluded that histopathology is not effective for distinguishing between BLE and LAD; accurate diagnosis relies on immunofluorescence and serological tests, even when clinical signs appear typical for one condition.
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Article Synopsis
  • * A case study of a full-term female infant suffering from NLABD highlights the condition's severe cutaneous and respiratory effects, with diagnosis confirmed through skin biopsy and immunofluorescence, revealing pathogenic IgAs in the mother's breastmilk.
  • * The infant successfully recovered after extensive care over 8 weeks, demonstrating the necessity for timely diagnosis and management, while presenting new insights into the role of maternal breastmilk in the disease's pathogenesis.
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Article Synopsis
  • Linear IgA bullous dermatosis (LABD) is an autoimmune skin disorder characterized by blistering and the presence of IgA deposits at the basement membrane.
  • Diagnosis involves skin pathology tests and direct immunofluorescence to confirm the diagnosis, with symptoms including tense blisters and intense itching.
  • Treatment often starts with dapsone, but in a reported case resistant to other therapies, dupilumab was used to manage itching while awaiting HLA-B*1301 gene test results to prevent serious drug reactions.
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Targeted serum proteome profiling reveals nicotinamide adenine dinucleotide phosphate (NADPH)-related biomarkers to discriminate linear IgA bullous disorder from dermatitis herpetiformis.

Clin Immunol

August 2024

Hospital for Skin Diseases, Shandong First Medical University, Jinan, Shandong, China; Shandong Provincial Institute of Dermatology and Venereology, Shandong Academy of Medical Sciences, Jinan, Shandong, China; National Clinical Key Project of Dermatology and Venereology, Jinan, Shandong, China. Electronic address:

Article Synopsis
  • Linear IgA bullous dermatosis (LABD) and dermatitis herpetiformis (DH) are types of autoimmune skin disorders linked to IgA, and this study aimed to explore their causes using serum proteomics.
  • Researchers analyzed 92 biomarkers in serum samples from LABD, DH patients, and healthy controls, discovering elevated levels of certain biomarkers specific to these conditions.
  • The study identified distinct biomarker clusters for LABD and DH, with six specific biomarkers showing potential for better prognosis in DH patients, which could help differentiate these disease subtypes in the future.
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State-of-the-art diagnosis of autoimmune blistering diseases.

Front Immunol

June 2024

Department of Dermatology, Allergology and Venerology, University of Lübeck, Lübeck, Germany.

Article Synopsis
  • - Autoimmune blistering disorders (AIBDs) include various conditions such as pemphigus and pemphigoid, characterized by blisters and skin lesions that can have diverse appearances, making accurate diagnosis essential for treatment and prognosis.
  • - Diagnosis typically combines clinical evaluation with the detection of specific autoantibodies, utilizing tests like enzyme-linked immunosorbent assay (ELISA) and direct immunofluorescence microscopy (IFM), which is considered the gold standard.
  • - Recent advancements in molecular identification of target antigens have led to new diagnostic methods and treatment approaches, enhancing the ability to detect specific antibodies related to various AIBDs.
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Article Synopsis
  • * A 45-year-old female patient with this condition experienced significant airway bleeding during a planned corneal transplant, leading to the involvement of multiple medical specialties for her care.
  • * Effective communication among the surgery, anesthesia, and dermatology teams was crucial for the patient's recovery, emphasizing that outpatient surgery is only advisable when the disease is in full remission and there is no mucous membrane involvement.
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Article Synopsis
  • Autoimmune bullous dermatosis (AIBD) cases have been observed in patients after receiving COVID-19 vaccinations, prompting a systematic review of their characteristics and outcomes.
  • The review analyzed 98 studies involving 229 new-onset AIBD cases (mostly bullous pemphigoid) and 216 flare cases (predominantly pemphigus), with mRNA vaccines being a common link.
  • Most AIBD cases emerged within 1 to 123 days after vaccination, with many patients experiencing favorable outcomes, highlighting the need for healthcare professionals to monitor post-vaccination effects carefully.
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Article Synopsis
  • Autoimmune vesiculobullous diseases (AIBDs) are characterized by skin blisters caused by autoantibodies, and the gold standard diagnostic test is direct immunofluorescence (DIF) on fresh-frozen tissue.
  • This study compared DIF using paraffin-embedded tissue (PE-DIF) to fresh-frozen tissue (FF-DIF) in 30 AIBD cases, finding that PE-DIF can detect similar patterns, particularly in pemphigus vulgaris and pemphigus foliaceous, although results can vary in intensity.
  • The research concluded that while PE-DIF can supplement diagnosis in situations where frozen tissues are unavailable, it tends to deteriorate faster than FF-DIF and should
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Article Synopsis
  • Linear IgA bullous dermatosis (LABD) is a rare autoimmune skin condition characterized by blisters caused by IgA autoantibodies, and can resemble bullous pemphigoid (BP), which is due to IgG and IgE autoantibodies.
  • A case study of a 67-year-old woman with metastatic ovarian cancer showed new blistering skin lesions after she started immunotherapy, which were diagnosed as LABD based on skin biopsy results.
  • The patient’s condition did not improve with prednisone but significantly healed after starting dapsone, emphasizing the need for accurate diagnosis through histology and immunofluorescence to guide effective treatment without halting immunotherapy.
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Article Synopsis
  • Linear IgA Dermatosis (LAD) is a rare autoimmune skin disease characterized by IgA deposits at the skin's basement membrane, affecting both children and adults with diverse clinical features.
  • The European Academy of Dermatology and Venereology (EADV) developed consensus guidelines by collaborating with 29 experts across multiple countries to ensure a comprehensive approach to diagnosis and treatment.
  • The resulting guidelines provide a combination of evidence-based and expert-based recommendations to aid dermatologists in effectively diagnosing and managing LAD.
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Linear IgA Bullous Dermatosis in Korea Using the Nationwide Health Insurance Database.

J Clin Med

February 2024

Department of Internal Medicine, College of Medicine, Yeungnam University, Daegu 42415, Republic of Korea.

Article Synopsis
  • Linear immunoglobulin A bullous dermatosis (LABD) is a rare autoimmune skin disease that causes blisters and is linked to certain medications and autoimmune disorders.
  • A study was conducted on 670 LABD cases in Korea from 2010 to 2022, revealing a higher incidence in people aged 60 and above, with an annual rate of 1.3 cases per 100,000 individuals.
  • The findings suggested that patients were typically treated with dapsone, had an average age of diagnosis around 56 years, and had a greater association of LABD with risk factors like malignancy and antibiotic use compared to other studies.
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