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Dermatologie (Heidelberg, Germany) Dec 2022Pemphigoid diseases comprise a heterogeneous group of subepidermal autoimmune blistering dermatoses characterized by autoantibodies against structural proteins of the... (Review)
Review
Pemphigoid diseases comprise a heterogeneous group of subepidermal autoimmune blistering dermatoses characterized by autoantibodies against structural proteins of the dermal-epidermal junction. Recent decades have witnessed a significant surge in the incidence of these diseases, which, in addition to general aging of the population, can be attributed to the availability of precise diagnostic methods and improved knowledge of the clinical and immunopathological spectrum. While bullous pemphigoid, mucous membrane pemphigoid, and linear IgA disease account for most pemphigoid disorders, less frequent, presumably underdiagnosed variants are increasingly becoming relevant for clinicians. These include epidermolysis bullosa acquisita, anti-p200 pemphigoid, pemphigoid gestationis, lichen planus pemphigoides, and recently defined entities such as IgM pemphigoid and Orf-induced pemphigoid. Accurate characterization and differentiation of these subtypes are not only of diagnostic relevance but may also be associated with therapeutic and prognostic implications for affected individuals. Due to the rarity of these diseases, no controlled prospective clinical trials currently exist, making their diagnosis and therapy challenging.
Topics: Humans; Pemphigoid, Bullous; Prospective Studies; Autoantibodies; Epidermolysis Bullosa Acquisita; Lichen Planus
PubMed: 37847383
DOI: 10.1007/s00105-023-05242-2 -
JAAD Case Reports Nov 2023
PubMed: 37842158
DOI: 10.1016/j.jdcr.2023.08.013 -
Clinical and Experimental Dermatology Oct 2019Pemphigoid diseases are autoimmune subepidermal blistering diseases affecting the skin and mucous membranes, which are caused by autoantibodies targeting structural... (Review)
Review
Pemphigoid diseases are autoimmune subepidermal blistering diseases affecting the skin and mucous membranes, which are caused by autoantibodies targeting structural hemidesmosomal proteins or hemidesmosome-associated proteins. Variants of pemphigoid can be differentiated based on targeted antigens and clinical aspects. In this review, we will discuss pemphigoid variants that predominantly affect the skin, and provide clinicians with clues to diagnosis.
Topics: Epidermolysis Bullosa Acquisita; Female; Humans; Linear IgA Bullous Dermatosis; Pemphigoid Gestationis; Pemphigoid, Bullous; Pregnancy
PubMed: 31099084
DOI: 10.1111/ced.13984 -
Frontiers in Immunology 2024Autoimmune blistering disorders (AIBDs) are a heterogeneous group of approximately a dozen entities comprising pemphigus and pemphigoid disorders and dermatitis... (Review)
Review
Autoimmune blistering disorders (AIBDs) are a heterogeneous group of approximately a dozen entities comprising pemphigus and pemphigoid disorders and dermatitis herpetiformis. The exact diagnosis of AIBDs is critical for both prognosis and treatment and is based on the clinical appearance combined with the detection of tissue-bound and circulating autoantibodies. While blisters and erosions on the skin and/or inspectable mucosal surfaces are typical, lesions may be highly variable with erythematous, urticarial, prurigo-like, or eczematous manifestations. While direct immunofluorescence microscopy (IFM) of a perilesional biopsy is still the diagnostic gold standard, the molecular identification of the major target antigens opened novel therapeutic avenues. At present, most AIBDs can be diagnosed by the detection of autoantigen-specific serum antibodies by enzyme-linked immunosorbent assay (ELISA) or indirect IFM when the clinical picture is known. This is achieved by easily available and highly specific and sensitive assays employing recombinant immunodominant fragments of the major target antigens, i.e., desmoglein 1 (for pemphigus foliaceus), desmoglein 3 (for pemphigus vulgaris), envoplakin (for paraneoplastic pemphigus), BP180/type XVII collagen (for bullous pemphigoid, pemphigoid gestationis, and mucous membrane pemphigoid), laminin 332 (for mucous membrane pemphigoid), laminin β4 (for anti-p200 pemphigoid), type VII collagen (for epidermolysis bullosa acquisita and mucous membrane pemphigoid), and transglutaminase 3 (for dermatitis herpetiformis). Indirect IFM on tissue substrates and in-house ELISA and immunoblot tests are required to detect autoantibodies in some AIBD patients including those with linear IgA disease. Here, a straightforward modern approach to diagnosing AIBDs is presented including diagnostic criteria according to national and international guidelines supplemented by long-term in-house expertise.
Topics: Humans; Autoantibodies; Autoimmune Diseases; Autoantigens; Skin Diseases, Vesiculobullous; Enzyme-Linked Immunosorbent Assay
PubMed: 38903493
DOI: 10.3389/fimmu.2024.1363032 -
Journal of the American Academy of... Jul 2023
Topics: Pregnancy; Female; Humans; Pruritus; Pemphigoid Gestationis; Pregnancy Complications
PubMed: 37187427
DOI: 10.1016/j.jaad.2023.05.016 -
The Journal of Dermatology May 2022
Topics: Female; Humans; Infant, Newborn; Pemphigoid Gestationis; Pemphigoid, Bullous; Pregnancy; Skin Diseases, Vesiculobullous
PubMed: 34939222
DOI: 10.1111/1346-8138.16291 -
BMC Veterinary Research Feb 2023Autoimmune subepidermal blistering diseases (AISBDs) are rare skin disorders of animals that were first identified in dogs but several AISBDs are now recognised in other... (Review)
Review
Autoimmune subepidermal blistering diseases (AISBDs) are rare skin disorders of animals that were first identified in dogs but several AISBDs are now recognised in other companion animal species. Most AISBDs in animals are homologues of the human diseases and are thought to share similar pathomechanisms of epidermal and/or mucosal blister formation caused by autoantibodies targeting structural proteins of the basement membrane zone (BMZ). Disruption of their structural function by the autoantibodies and/or recruited inflammation leads to BMZ fragility, which presents clinically as vesicles, bullae and, later, deep erosions and ulcers. Canine AISBDs are the best characterised, particularly the more common variants such as mucous membrane pemphigoid (48%), epidermolysis bullosa acquisita (EBA) (26%), and bullous pemphigoid (10%). Exceedingly rare AISBDs in the dog are junctional EBA, mixed AISBD, type-1 bullous systemic lupus erythematosus, linear IgA dermatosis, and pemphigus gestationis. The diagnosis of a specific AISBD is made by combining the clinical features (breed, age, lesion distribution) with histological evidence of subepithelial clefting, but not all AISBDs can be differentiated in this manner and specialised immunological testing is required. This latter, unfortunately, is not readily available and, therefore, the specific AISBD diagnosis often remains unconfirmed. While this limits further understanding of these diseases, it does not prevent clinicians from treating their patients, as the treatment approaches are similar for the different AISBDs in dogs. This review primarily focuses on canine AISBDs, the species for which these diseases have been best characterised, and shorter descriptions of variants in other species are also provided.
Topics: Humans; Animals; Dogs; Skin; Pemphigus; Epidermis; Autoantibodies; Breeding; Dog Diseases
PubMed: 36849885
DOI: 10.1186/s12917-023-03597-1 -
Cureus Nov 2023Pemphigoid gestationis (PG) is a rare autoimmune bullous disease that occurs during pregnancy or the postpartum period. PG has been associated with an increased risk of...
Pemphigoid gestationis (PG) is a rare autoimmune bullous disease that occurs during pregnancy or the postpartum period. PG has been associated with an increased risk of Graves' disease possibly due to shared genetic factors and immune system fluctuations during pregnancy. However, the evidence supporting the association between PG and Graves' disease is mixed. Although dermatologists are cautioned to watch for Graves' disease in patients with a history of PG, this guidance is based on a single cohort where most patients were diagnosed with Graves' disease prior to PG onset. Recent data failed to find an association between Graves' disease and PG but did not capture the lifetime risk of Graves' disease in these patients. Future studies could focus on long-term follow-up of females with PG, shedding light on the lifetime risk profiles of these patients.
PubMed: 38106729
DOI: 10.7759/cureus.48972 -
Journal of the American Academy of... Jul 2021Subepithelial autoimmune blistering dermatoses are a group of rare skin disorders that are characterized by the disruption of the dermal-epidermal junction through the... (Review)
Review
Subepithelial autoimmune blistering dermatoses are a group of rare skin disorders that are characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The third article in this continuing medical education series explores the background, epidemiology, clinical features, and diagnostic criteria of each of the major subepithelial autoimmune blistering dermatoses, including bullous pemphigoid, pemphigoid gestationis, lichen planus pemphigoides, mucous membrane pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis.
Topics: Autoimmune Diseases; Dermis; Female; Humans; Lichen Planus; Pemphigoid Gestationis; Pregnancy; Skin Diseases, Vesiculobullous
PubMed: 33684496
DOI: 10.1016/j.jaad.2020.11.076 -
Cutis May 2023
Topics: Female; Humans; Pregnancy; COVID-19; COVID-19 Vaccines; Pemphigoid Gestationis; Pemphigoid, Bullous; Vaccination
PubMed: 37406312
DOI: 10.12788/cutis.0772