194 research outputs found
Table 1 for Multidisciplinary Care of Epidermolysis Bullosa during the COVID-19 Pandemic – Consensus: Recommendations by an International Panel of Experts
No full textA questionnaire was drafted by DM into a table of suggested modifications to the management of EB during the COVID-19 pandemic. Fifty seven well-known experts on EB were selected based on membership of the international Clin-et group and/or clinical expertise in EB demonstrated at International EB Congress participation. Responses and reasons for each response were requested individually to the lead author based on an ideal scenario, rather than what actually may happen in some centers with financial constraints. A priori, consensus was considered to be the agreement of more than 70% of respondents with the suggestion. Questionnaires were returned by 44 of the 57 EB experts, representing several areas of clinical expertise in EB (dermatology, pediatrics, internal medicine and surgery) from 5 continents. After addition and revision of some items and 3 cycles of revoting, consensus was achieved for all items, which are summarized in this Table. The full details are published in the JAAD -doi: 10.1016/j.jaad.2020.06.102
Beyond the skin: disease parameters in pemphigus
No full textPemphigus represents a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. This group has a chronic course leading to high morbidity and mortality. Because of the painful chronic-recurring blisters and/or erosions on skin and mucosa, pemphigus can impair quality of life (QOL). Therapeutic modalities, anxiety and depression can also have an additional negative impact in the QOL of the pemphigus patients. Since the nature and course of the pathology and the fact that pemphigus worsens the quality of life of affected patients, scoring systems to objectively evaluate the clinical activity of the disease and to correlate that with the QOL are needed. Nowadays the most used global scales to assess the clinical activity of pemphigus are the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), the Pemphigus Disease Area Index (PDAI) and the Pemphigus Visual Activity Scale (PVAS). To evaluate the patient's generic QOL the most used score is the Dermatology Life Quality Index (DLQI), but all the sponsered clinical trials in pemphigus are using ABQOL this rather than DLQI
Flightless I over-expression impairs skin barrier development, function and recovery following skin blistering
No full textAbstract not availableZlatko Kopecki, Gink N Yang, Ruth M Arkell, Jessica E Jackson, Elizabeth Melville, Hiroaki Iwata, Ralf J Ludwig, Detlef Zillikens, Dedee F Murrell and Allison J Cowi
Bullous pemphigoid: therapeutic algrorithm and practical management
No full textIntroduction: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucosae. BP typically affects the elderly and manifests with severe itch, localised or generalised eczematous, urticated and/or bullous lesions. Its morbidity and impact on the quality of life are important. The disease is significantly associated with neurological disorders, such as stroke, Parkinson disease, major cognitive impairment and multiple sclerosis. Diagnosis of BP critically relies on immunopathologic examinations, particularly direct immunofluorescence microscopy studies.
Areas covered: This paper looks at the evidence of therapies commonly used in bullous pemphigoid.
Expert opinion: Treatment of BP has been a challenge, given the relative rarity of the disease, lack of good quality randomised controlled trials, the presence of co-morbidities in the affected elderly population and the high mortality rate. Recent controlled studies have indicated that potent topical corticosteroids constitute a more effective therapy for BP when compared to oral corticosteroids in terms of control of the disease, side effect profile and overall survival. Other therapies have been employed with varying success, but are not validated yet. Improved knowledge of the pathophysiology of BP will hopefully allow the development of new immunomodulatory treatments for this debilitating disease
A lesson learned about predatory journals and their difference from peer-reviewed open-access publishing
No full textTopically applied flightless I neutralizing antibodies improve healing of blistered skin in a murine model of Epidermolysis Bullosa Acquisita
No full textData source: supplementary material, https://doi.org/10.1038/jid.2012.457Epidermolysis bullosa (EB) is a chronic inheritable disease that leads to severe blistering and fibrosis. Previous studies have shown that the actin cytoskeletal protein flightless I (Flii) impairs wound healing associated with EB. Using a mouse model of EB acquisita (EBA), the effect of ‘‘mopping up’’ Flii using Flii-neutralizing antibodies (FnAbs) before, during, and after blister formation was determined. FnAbs, incorporated into a cream vehicle and applied topically to the skin, penetrated into the basal epidermis and upper papillary dermis but were not detected in serum or other organs and did not alter neutrophil or macrophage infiltration into the blistered skin. Histological assessment of blister severity showed that treatment of early-stage blisters with FnAb cream reduced their severity and improved their rate of healing. Treatment of established blisters with FnAb cream also improved healing and restored the skin’s tensile strength toward that of normal skin. Repeated application of FnAbs to EBA skin before the onset of blistering reduced the severity of skin blistering. Independent of when the FnAbs were applied, skin barrier function and wound healing were improved and skin fragility was reduced, suggesting that FnAbs could potentially improve healing of patients with EB.Zlatko Kopecki, Nadira Ruzehaji, Christopher Turner, Hioraki Iwata, Ralf J. Ludwig, Detlef Zillikens, Dedee F. Murrell and Allison J. Cowi
Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management.
No full textAutoimmune subepidermal blistering diseases of the skin and mucosae constitute a large group of sometimes devastating diseases, encompassing bullous pemphigoid, gestational pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and anti-p200 pemphigoid. Their clinical presentation is polymorphic. These autoimmune blistering diseases are associated with autoantibodies that target distinct components of the basement membrane zone of stratified epithelia. These autoantigens represent structural proteins important for maintenance of dermo-epidermal integrity. Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disease of the skin and mucosae. Although the disease typically presents with a generalized blistering eruption associated with itch, atypical variants with either localized bullous lesions or "non-bullous" presentations are observed in approximately 20% of patients. A peculiar form of BP typically associated with pregnancy is pemphigoid gestationis. In anti-p200 pemphigoid, patients present with tense blisters on erythematosus or normal skin resembling BP, with a predilection for acral surfaces. These patients have antibodies targeting the 200-kDa basement membrane protein. Epidermolysis bullosa is a rare autoimmune blistering disease associated with autoantibodies against type VII collagen that can have several phenotypes including a classical form mimicking dystrophic epidermolysis bullosa, an inflammatory presentation mimicking BP, or mucous membrane pemphigoid-like lesions. Mucous membrane pemphigoid (MMP) is the term agreed upon by international consensus for an autoimmune blistering disorder, which affects one or more mucous membrane and may involve the skin. The condition involves a number of different autoantigens in the basement membrane zone. It may result in severe complications from scarring, such as blindness and strictures. Diagnosis of these diseases relies on direct immunofluorescence microscopy studies and immunoserological assays. Management of affected patients is often challenging. We will here review the clinical and immunopathological features as well as the pathophysiology of this group of organ-specific autoimmune diseases. Finally, we will discuss the diagnostic approach and the principles of management in clinical practice
Skin Blistering Diseases
No full textThis eBook is a collection of articles from a Frontiers Research Topic. Frontiers Research Topics are very popular trademarks of the Frontiers Journals Series: they are collections of at least ten articles, all centered on a particular subject. With their unique mix of varied contributions from Original Research to Review Articles, Frontiers Research Topics unify the most influential researchers, the latest key findings and historical advances in a hot research area! Find out more on how to host your own Frontiers Research Topic or contribute to one as an author by contacting the Frontiers Editorial Office: frontiersin.org/about/contac
Lupus profundus limited to a site of trauma: Case report and review of the literature
No full textLupus erythematosus profundus (LEP) is a rare form of chronic cutaneous lupus erythematosus. We report on a case of a 56-year-old Caucasian woman who presented with a single, persistent, painful rash on the left hip and lateral aspect of the left upper thigh, which had been present for 2.5 years. The patient had a history of previous injury to this area before the rash started. Clinical findings showed an inflamed, hyperpigmented, and indurated plaque with a linear skin invagination and no associated systemic symptoms. A skin biopsy test result confirmed the diagnosis of LEP and the clinical and laboratory examinations ruled out systemic lupus erythematosus. After 2 months of treatment with methotrexate 20 mg weekly and 1 month of prednisolone 7.5 mg daily, the skin rash improved considerably. We also present a brief review of the epidemiology, etiology, clinical features, histopathology, laboratory findings, differential diagnosis, and treatment of LEP.Versión Publicad
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