Autoimmune blistering dermatoses in children.

[Autoimmune blistering dermatoses in children]. Hautarzt. 2019 Apr 02;: Authors: Kiritsi D, Schauer F Abstract Autoimmune blistering skin disorders represent a rare group of autoantibody-induced dermatoses against desmosomal and hemidesmosomal molecules. The common age of onset for pemphigus and pemphigoid, as well as dermatitis herpetiformis, encompasses the adult age, but all these disorders can be observed neonatally and/or during childhood. If the disease occurs postpartum or neonatally, physicians should consider transplacental transmission of pathogenic maternal immunoglobulin G (IgG)-autoantibodies, and both mother and child should be included in the diagnostic work up. If the disorder is suspected in childhood, early immunoserological testing and skin biopsies for direct immunofluorescence analyses are recommended for the correct diagnosis and subsequently for the right choice of treatment. First-line recommendations are nonhalogenated topical steroids, followed by oral dapsone. All therapies require preliminary examinations, e. g. enzyme-activity testing (as is glucose-6-phophate dehydrogenase in dapsone treatment). In refractory cases, further treatment choices like high-dose intravenous immunoglobins, plasmapheresis/immunoadsorption or targeted therapies like anti-CD20 autoantibody therapies are indicated. An intense dermatological support and good medical care are essential for an age-appropriate development of the child and to lower po...
Source: Der Hautarzt: Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete - Category: Dermatology Tags: Hautarzt Source Type: research

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Autoimmune bullous dermatoses (AIBD) are characterized by circulating autoantibodies that are either directed against epidermal antigens or deposited as immune complexes in the basement membrane zone. The complement system (CS) can be activated by autoantibodies, thereby triggering activation of specific complement pathways. Local complement activation induces a pathogenic inflammatory response that eventually results in the formation of a sub- or intraepidermal blister. Deposition of complement components is routinely used as a diagnostic marker for AIBD. Knowledge from different animal models mimicking AIBD and depositio...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Conclusions The clinical trials discussed here, which include several trials investigating novel therapeutic targets, demonstrate that translational research in pemphigus and pemphigoid is a fast-growing field. We thus expect that several novel treatments will be shortly available for the treatment of pemphigus and pemphigoid patients. Given the high, and thus far unmet, medical need in this field (110), this is highly encouraging and will hopefully improve the quality of life of the affected patients. In addition to the compounds and targets described here, several new targets have been recently identified in preclinical...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Psoriasis (PSO), atopic dermatitis or eczema (ADE), pemphigus, bullous pemphigoid (BP), hidradenitis (HS) and dermatomyositis (DM) are chronic inflammatory skin disorders (CISD) associated with systemic immune activation and dysregulation, and possible autoimmune disease. We sought to determine whether these CISD are associated with increased risk of comorbid autoimmune disease. Data from the 20022012 National Inpatient Sample were analyzed, including a representative 20% sample of all US hospitalizations (n=72,108,077 adults).
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Clinical Research: Epidemiology of Skin Diseases Source Type: research
Autoimmune blistering diseases (AIBD) are a group of chronic diseases affecting the skin and mucous membranes, with different presentation, clinical course, histologic and immunopathologic findings, and different therapeutic approach. Blisters develop as a result of autoantibodies directed against distinct adhesion structures within desmosomes or within the basement membrane zone. The most common AIBD that develops in the elderly is bullous pemphigoid (previously also named “pemphigoid senilis”), but mature patients can also present with other AIBD as mucous membrane pemphigoid, epidermolysis bullosa acquisita,...
Source: Clinics in Dermatology - Category: Dermatology Authors: Source Type: research
ConclusionsAutoimmune bullous diseases present characteristic trichoscopic patterns. Trichoscopy can be regarded as a rapid in‐office preliminary diagnostic method in the differential diagnosis of these diseases.
Source: International Journal of Dermatology - Category: Dermatology Authors: Tags: Report Source Type: research
This study was conducted to determine the clinicoepidemiologic features of ABDs in elderly patients. Medical records of all ABD patients with disease onset after the age of 60 years who presented to the Autoimmune Bullous Diseases Research Center, Tehran, Iran between April 2003 and March 2013 were reviewed. Patients with dermatitis herpetiformis were not included. During the 10-year period studied, 296 patients with ABD and disease onset after 60 years of age were diagnosed. Bullous pemphigoid (BP) was observed to be the most common ABD (48.3%), followed by pemphigus vulgaris (45.3%), pemphigus foliaceus (3.7%), mucous me...
Source: Skinmed - Category: Dermatology Authors: Tags: Skinmed Source Type: research
CONCLUSION: Evaluation of triggering factors and comorbidities before starting treatment may assist in controlling the disease more swiftly and reducing the side effects of treatment in the management of ABDs. However, to evaluate the frequency and significance of our findings in order to determine whether they are statistically significant or not, we suggest a multicenter prospective clinical trial, which would include control groups and a higher number of patients for each of the disease groups and clinical subtypes. PMID: 28618730 [PubMed - in process]
Source: Turkish Journal of Medical Sciences - Category: General Medicine Tags: Turk J Med Sci Source Type: research
Autoimmune blistering diseases are a heterogeneous group of about a dozen complex disorders that are characterized by intraepidermal (pemphigus) and subepidermal blistering (pemphigoid diseases and dermatitis herpetiformis). The Pathogenesis of Pemphigus and Pemphigoid Meeting, organized by the Departments of Dermatology in L übeck and Marburg and the Institute of Anatomy and Cell Biology, Munich, was held in September 2016 in Munich. The meeting brought together basic scientists and clinicians from all continents dedicating their work to autoimmune blistering diseases.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Meeting Report Source Type: research
ConclusionPAIBD are associated with a considerable inpatient burden and comorbid health conditions.
Source: American Journal of Clinical Dermatology - Category: Dermatology Source Type: research
Authors: Sobhan M, Farshchian M, Tamimi M Abstract BACKGROUND: Autoimmune bullous diseases (ABDs) represent a group of rare, acquired disorders characterized by overlapping features with involvement of the skin and mucous membranes, resistance to treatment, and potential lethality that comprise pemphigus, bullous pemphigoid (BP), epidermolysis bullosa, dermatitis herpetiformis, and linear immunoglobulin A bullous dermatosis. AIM: The main aim of this study was to identify the epidemiologic characteristics and clinical courses of these common diseases in Hamadan, Iran. Few surveys have been carried out to demons...
Source: Clinical, Cosmetic and Investigational Dermatology - Category: Dermatology Tags: Clin Cosmet Investig Dermatol Source Type: research
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