Antibody reactive to a novel autoantigen on the nondesmosomal keratinocyte surfaces leads to herpetiform pemphigus.
Antibody reactive to a novel autoantigen on the nondesmosomal keratinocyte surfaces leads to herpetiform pemphigus. Br J Dermatol. 2019 Jan;180(1):22 Authors: Yuan H, Pan M PMID: 30604537 [PubMed - in process]
Condition: Pemphigus Vulgaris Interventions: Drug: recombinant human interleukin-2 (rhIL-2); Drug: placebo Sponsor: Second Xiangya Hospital of Central South University Not yet recruiting
AbstractAutoimmune bullous diseases are a group of chronic inflammatory disorders caused by autoantibodies targeted against structural proteins of the desmosomal and hemidesmosomal plaques in the skin and mucosa, leading to intra-epithelial or subepithelial blistering. The oral mucosa is frequently affected in these diseases, in particular, in mucous membrane pemphigoid, pemphigus vulgaris, and paraneoplastic pemphigus. The clinical symptoms are heterogeneous and may present with erythema, blisters, erosions, and ulcers localized anywhere on the oral mucosa, and lead to severe complaints for the patients including pain, dy...
Few large-scale and controlled studies examined the associations and predictors of the gamut of autoimmune conditions in pemphigus and pemphigoid.
This study builds on previous case reports of cutaneous disorders in Castleman's disease and proposes a new classification system. PMID: 31282978 [PubMed - as supplied by publisher]
PMID: 31282977 [PubMed - as supplied by publisher]
Conclusion: Immunohistochemical labeling for IgG4 on paraffin-embedded tissue provides a sensitive and specific test for diagnosing pemphigus in a situation when fresh frozen tissue is unavailable.
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...
Pemphigus encompasses a heterogeneous group of autoimmune blistering diseases, which affect both mucous membranes and the skin. The disease usually runs a chronic-relapsing course, with a potentially devastating impact on the patients’ quality of life. Pemphigus pathogenesis is related to IgG autoantibodies targeting various adhesion molecules in the epidermis, including desmoglein (Dsg) 1 and 3, major components of desmosomes. The pathogenic relevance of such autoantibodies has been largely demonstrated experimentally. IgG autoantibody binding to Dsg results in loss of epidermal keratinocyte adhesion, a phenomenon r...