774 results match your criteria: "Pemphigus Herpetiformis"

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.
View Article and Find Full Text PDF

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.
View Article and Find Full Text PDF

A Critical Appraisal of Gluten in Skin Disorders, the Evidence So Far, and Updated Recommendations.

Indian J Dermatol

April 2024

From the Department of Dermatology, Venereology and Leprosy, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.

Article Synopsis
  • Gluten, a protein found in cereals like wheat and barley, has been linked to various skin disorders including psoriasis and urticaria, although research on this connection is not very thorough.
  • A comprehensive search of medical databases identified 1487 articles, ultimately narrowing it down to 114 relevant studies, which were reviewed to assess gluten's role in these conditions.
  • While a gluten-free diet may help some chronic skin issues, it's important to note that in most cases, gluten restriction isn’t necessary and could potentially cause nutritional deficiencies.
View Article and Find Full Text PDF
Article Synopsis
  • - Pemphigus herpetiformis (PH) is a rare autoimmune skin condition that mostly affects adults, but it's extremely uncommon in young children, like the three-year-old girl in the case study.
  • - The child exhibited skin issues resembling other conditions, and a biopsy revealed several specific features, including inflammation and blisters.
  • - Treatment with clobetasol ointment and dapsone led to significant improvement in her symptoms, highlighting the need to recognize PH in pediatric cases and encouraging more research to understand it better.
View Article and Find Full Text PDF
Article Synopsis
  • A bibliometric analysis was conducted on Pemphigus research published between 1980 and 2021, revealing a total of 3034 articles analyzed for trends, collaborations, and effective studies.
  • The USA, Japan, and Germany were identified as the top contributors to Pemphigus literature, with significant outputs from institutions like Keio University, Kurume University, and Tel Aviv University.
  • Key topics explored included various types of Pemphigus, treatment methods like rituximab, and factors impacting quality of life, with pemphigus vulgaris and pemphigus foliaceus being the most commonly studied.
View Article and Find Full Text PDF
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
View Article and Find Full Text PDF

The Need of Differential Diagnosis Between Vulvar Lichen Sclerosus and Autoimmune Dermatoses in Adolescent Girls.

Dermatol Ther (Heidelb)

March 2024

Chair and Department of Gynecology, Obstetrics and Oncological Gynecology, Faculty of Medical Sciences in Katowice, Medical University of Silesia in Katowice, Markiefki 87, 40-211, Katowice, Poland.

Article Synopsis
  • Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition primarily affecting the vulvar area in young girls, causing symptoms like itching, discomfort, and a characteristic 'figure 8' appearance during exams.
  • The study aimed to differentiate VLS from other autoimmune skin diseases by analyzing medical histories and blood samples of affected girls aged 2-18.
  • Key findings showed that symptoms like itching, soreness, and erythema were prevalent in the VLS group, highlighting the need for proper diagnosis and management to improve patient care.
View Article and Find Full Text PDF
Article Synopsis
  • * Common issues include pain, taste disturbance, and dysphagia, negatively impacting patients' quality of life and leading to potential delays in correct diagnosis and treatment.
  • * This review serves as an evidence-based reference for clinicians, covering key conditions, their clinical features, diagnostic methods, and treatment options, divided into two parts focusing on different disorders.
View Article and Find Full Text PDF
Article Synopsis
  • Autoimmune bullous diseases (ABDs) are rare skin conditions characterized by autoantibodies affecting the skin, posing challenges for dermatologists, especially in Romania where there is limited data on their prevalence.
  • A study from 2015 to 2019 analyzed 225 patients in northeastern Romania, finding pemphigus to be the most common ABD with an incidence of 8.16 per million, followed by bullous pemphigoid and dermatitis herpetiformis.
  • The findings indicated that pemphigus vulgaris occurs more frequently in this region compared to Western Europe, potentially due to unique geographical and genetic factors affecting the local population.
View Article and Find Full Text PDF
Article Synopsis
  • - *Pemphigus herpetiformis (PH) is a skin disorder characterized by blisters and erosions, notably affecting a 61-year-old female patient who experienced itchy lesions and hair loss, while her oral and genital areas remained unaffected.* - *Histopathological examination showed specific skin changes, including thinning of the epidermis and certain inflammatory cell patterns, along with direct immunofluorescence revealing IgG and IgA deposits in a unique pattern.* - *The diagnosis of PH was confirmed, and the patient showed significant improvement with treatment using dapsone and steroids.*
View Article and Find Full Text PDF
Article Synopsis
  • HLA class II molecules are important in determining susceptibility to autoimmune disorders, especially in skin conditions like psoriasis, but their role in antibody-mediated skin diseases is less understood.
  • Research summarized various HLA alleles associated with different blistering skin diseases, highlighting risk alleles like HLA-DQB1*0503 and HLA-DRB1*0402 in pemphigus, HLA-DQB1*0301 in pemphigoid, and strong associations with HLA-DQ2 and HLA-DQ8 in dermatitis herpetiformis.
  • Understanding these HLA associations can help in personalized medicine approaches by identifying at-risk individuals who may need to avoid specific disease triggers.
View Article and Find Full Text PDF

[Trigger factors associated with bullous autoimmune dermatoses].

Dermatologie (Heidelb)

December 2022

Klinik für Dermatologie, Allergologie und Venerologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.

Article Synopsis
  • Blistering autoimmune dermatoses are rare diseases that include pemphigus and pemphigoid, with the former causing blisters due to a loss of adhesion between skin cells, and the latter due to a loss of adhesion from the basement membrane.
  • Key triggers for these conditions include vaccinations, viral infections, UV exposure, and certain medications, with age being a significant risk factor for bullous pemphigoid.
  • Understanding these triggers can help diagnose these conditions early and aid in preventing relapses.
View Article and Find Full Text PDF
Article Synopsis
  • The oral mucosa can be affected by various immune-mediated conditions that cause inflammation and pain, impacting patients' oral health and quality of life.
  • Many of these conditions have overlapping symptoms, leading to potential delays in accurate diagnosis and appropriate treatment.
  • This review serves as a comprehensive resource for clinicians, detailing the oral manifestations, clinical features, and recent research on immune-mediated mucocutaneous conditions, focusing on specific disorders over two parts.
View Article and Find Full Text PDF
Article Synopsis
  • Autoimmune bullous diseases (AIBDs) are rare skin disorders that result in blistering and erosions caused by autoantibodies targeting structural proteins in the skin, and they can lead to serious complications if untreated.
  • The study focused on the clinical and epidemiologic features of AIBDs in Middle Franconia, involving 242 patients diagnosed or treated at a university hospital over six years, with data analyzed using Microsoft Excel.
  • The results highlight the incidence of various types of AIBDs, including pemphigus vulgaris and bullous pemphigoid, demonstrating advances in diagnosis and treatment options beyond traditional corticosteroids.
View Article and Find Full Text PDF
Article Synopsis
  • * Conducted at the University of Utah Health, researchers analyzed Skindex-16 scores from 192 patients diagnosed with various AIBD subtypes between 2016 and 2020, assessing disease severity and patient-reported flare status.
  • * Results showed that flare state scores were notably higher, indicating a greater negative effect on skin-related quality of life, and the study also evaluated how well Skindex-16 correlated with medical and patient-reported outcomes.
View Article and Find Full Text PDF
Article Synopsis
  • - The study evaluated high-dose intravenous immunoglobulin (IVIG) therapy for treating autoimmune bullous diseases (AIBDs), focusing on clinical remission, glucocorticosteroid reduction, and safety over 12 months.
  • - A group of 10 patients with various types of AIBDs received IVIG infusions, resulting in significant reductions in prednisone dosage and overall improvements in disease status; however, 20% of patients experienced relapse.
  • - While IVIG therapy showed effectiveness and an acceptable safety profile with mostly mild side effects, its high cost poses challenges for accessibility, especially in low-income areas, making it a consideration for patients who can't use standard treatments.
View Article and Find Full Text PDF