Pemphigoid variants affecting the skin

Lamberts, Aniek; Rashid, Hanan; Pas, Hendrikus; Diercks, Gilles F.H.; Meijer, Joost; Hórvath, Barbara

doi:10.1111/ced.13984

Cited by 4 publications

(2 citation statements)

References 33 publications

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“…1 Pemphigoid diseases driven solely by immunoglobulin A (IgA) autoantibodies are generally classified as linear IgA bullous dermatosis (LABD) or linear IgA disease (LAD) regardless of the antigen specificity. 2,3 In the literature, a lamina lucida subtype and a sublamina densa subtype of LABD are distinguished 4,5 by IgA autoantibodies to the soluble 120 kDa ectodomain of BP180, LAD-1, 6 or to type VII collagen (COL7), respectively. 5,7 The importance of this difference in autoantigen specificity for the clinical phenotype is a matter of debate.…”

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confidence: 99%

Evaluation and Comparison of Clinical and iLaboratory Characteristics of Patients With IgA Epidermolysis Bullosa Acquisita, Linear IgA Bullous Dermatosis, and IgG Epidermolysis Bullosa Acquisita

et al. 2021

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IMPORTANCE Immunoglobulin A (IgA) epidermolysis bullosa acquisita (EBA) is an autoimmune skin blistering disease with IgA autoantibodies directed against type VII collagen. There is debate whether it should be considered part of the clinical spectrum of linear IgA bullous dermatosis (LABD) or a separate disease entity.OBJECTIVE This cohort study aimed to define the clinical features and treatment responses of IgA EBA and anti-BP180-driven LABD, and to compare the prevalences of IgA EBA anti-BP180 LABD and classic IgG-mediated EBA in an autoimmune diagnostic laboratory database. DESIGN, SETTING, AND PARTICIPANTSThis retrospective cohort study and case series study included demographic, immunopathologic, and serologic data from 300 patients diagnosed with IgA EBA, IgG EBA, or LABD. Furthermore, clinical features and treatment responses of IgA EBA were analyzed in a case series including 4 patients with IgA EBA. All patients from the database of the autoimmune diagnostic laboratory at the

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confidence: 99%

Evaluation and Comparison of Clinical and iLaboratory Characteristics of Patients With IgA Epidermolysis Bullosa Acquisita, Linear IgA Bullous Dermatosis, and IgG Epidermolysis Bullosa Acquisita

et al. 2021

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“…Linear IgA disease is a rare AISBD described in only two adult dogs (3% of all AISBDs); one cross-bred Labrador retriever (3-year-old, female spayed) and one briard (4-year-old, male neutered) [ 2 , 5 ]. Interestingly, human LAD exhibits a biphasic age distribution affecting both young children (age 6 months to 6 years) and older adults (> 60 years) [ 60 , 63 ].…”

Section: Linear Iga Disease (Lad or Linear Iga Bullous Dermatosis (La...mentioning

confidence: 99%

Spontaneous autoimmune subepidermal blistering diseases in animals: a comprehensive review

et al. 2023

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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.

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