1,100 results match your criteria: "Epidermolysis Bullosa Acquisita"

We present the case of a 36-year-old paraplegic woman with a history of spinal cord injury who developed a generalized blistering rash, later diagnosed as bullous pemphigoid (BP). During her hospitalization, she was treated with prednisone and rituximab infusions, transitioning to maintenance therapy with topical steroids, doxycycline, and nicotinamide. A year later, she presented with concerns about a BP flare on her feet.

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Milia within resolving bullous pemphigoid lesions.

Dermatol Online J

August 2024

Department of Dermatology, King Abdullah Medical Complex, Jeddah, Saudi Arabia.

Article Synopsis
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Gastrointestinal (GI) diseases can present with several extraintestinal manifestations, and cutaneous signs and symptoms are most frequent. Although conventionally GI and skin are considered two entirely separate organ systems, they are closely correlated in origin. An increasing amount of data highlights the complex relationship between GI and dermatological conditions.

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Article Synopsis
  • Autoimmune blistering diseases (AIBDs), such as bullous pemphigoid (BP) and pemphigus vulgaris (PV), cause severe skin and mucosal blistering but are under-researched in Japan due to their rarity and limited medical coverage for less severe cases.
  • The study analyzed data from a nationwide database, identifying significant case numbers for different AIBDs, with findings showing that BP patients are generally older and have higher comorbidities compared to other AIBDs.
  • In-hospital mortality rates were notably higher for BP and epidermolysis bullosa acquisita (EBA) than for PV and pemphigus foliaceous (PF), with age being a critical risk factor for mortality across
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Background: Epidermolysis bullosa acquisita (EBA) is a chronic mucocutaneous disease that is mediated by antibodies that bind collagen VII. The treatment of EBA can be challenging and often multiple immunomodulatory drugs are required. Rituximab has been reported to be an effective treatment for recalcitrant EBA, although its evidence base is limited to case reports and case series.

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Current Knowledge on Nail Involvement in Autoimmune Bullous Disorders.

Skin Appendage Disord

October 2024

Dermatology Departement, Saint Pierre and Brugmann University Hospitals, Université Libre de Bruxelles, Brussels, Belgium.

Article Synopsis
  • Nail involvement in autoimmune bullous disorders presents diverse symptoms, often differing from skin manifestations, such as paronychia, onychomadesis, and onycholysis without blisters.
  • Various autoimmune conditions like pemphigus vulgaris and bullous pemphigoid can cause nail changes, and the presence of Langerhans cells in the nails is noteworthy due to its unique immunological aspects.
  • Diagnosing these nail issues typically requires histopathological and immunofluorescence tests, and researchers stress the need for more studies to improve treatment strategies for affected individuals.
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  • - The autoimmune skin disease epidermolysis bullosa acquisita (EBA) is driven by autoantibodies against type VII collagen, leading to severe blistering, and is influenced by immune cells and proteases like granzyme B (GzmB).
  • - Researchers tested a new GzmB inhibitor, SNT-6935, and found that it notably reduced skin damage caused by anti-COL7 antibodies in a lab model of EBA.
  • - The study suggests that while GzmB plays a crucial role in EBA's damage mechanism, other factors may also contribute, supporting GzmB as a potential treatment target for EBA and similar diseases.
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  • * Methods: Researchers conducted a thorough review of literature from various medical databases to gather relevant studies on RTX's effectiveness and safety for treating EBA.
  • * Results: Out of 31 studies reviewed, RTX showed positive outcomes with a high clinical response (92.7%) and disease remission (73.8%), though a notable relapse rate (39.5%) was observed, with mild side effects experienced by some patients.
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[Discovering the pathogenesis of autoimmune inflammation].

Orv Hetil

June 2024

1 Semmelweis Egyetem, Általános Orvostudományi Kar, Élettani Intézet Budapest, Üllői út 26., 1085 Magyarország.

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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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  • - Epidermolysis bullosa acquisita (EBA) is an autoimmune skin disease caused by autoantibodies against type VII collagen, making its treatment challenging with a long median remission time of 9 months.
  • - Research shows that removing regulatory T cells increases inflammation and blistering in EBA models, and the role of interferon gamma (IFN-γ) in this process was previously unclear.
  • - Treatment with an anti-IFN-γ antibody resulted in significant clinical improvement of EBA symptoms and reduced inflammation, suggesting that targeting IFN-γ could be a viable therapeutic approach for EBA and similar conditions.
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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.
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  • Rituximab (RTX) is primarily used to treat autoimmune bullous diseases (AIBDs), and this research evaluated its benefits and safety in patients over a year-long period.
  • In the study involving 11 patients with various AIBDs, significant reductions in steroid (prednisone) use and autoantibody levels were observed following RTX treatment.
  • While 18% of patients had disease relapses, most achieved remission with minimal therapy, and adverse effects were mild, indicating RTX is a promising option for managing refractory AIBD, though further research on dosage and maintenance is needed.
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Clinical Features of Paediatric Inflammatory Epidermolysis Bullosa Acquisita: A Case Series Study.

Acta Derm Venereol

January 2024

Department of Dermatology, Peking University First Hospital, Beijing, China; National Clinical Research Center for Skin and Immune Diseases, Beijing, China; Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing, China.

Article Synopsis
  • The study focuses on epidermolysis bullosa acquisita (EBA), a rare autoimmune skin condition that occurs infrequently in children, examining 7 pediatric patients aged 16 and under.
  • All patients showed inflammatory EBA, with some displaying symptoms similar to bullous pemphigoid, and unique histopathological features such as specific neutrophil distributions.
  • Treatment involved glucocorticoids and other medications like dapsone, thalidomide, and sulfasalazine, all of which were effective, but relapses often happened when glucocorticoid dosages were reduced or stopped, indicating a need for tailored treatment and follow-up for children with this condition.
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  • * Researchers used advanced imaging techniques and specific mouse models to track how different monocyte subpopulations infiltrate inflamed skin, particularly in epidermolysis bullosa acquisita (EBA) triggered by a specific antibody.
  • * Findings showed that monocytes recruit similarly to inflamed skin in various reporter mice, and even when lacking key chemokine receptors (CCR2 and CX3CR1), the recruitment process and disease severity were not significantly altered.
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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.
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