Abstract
The presence of anti-desmocollin (Dsc) antibodies is rarely described in autoimmune blistering diseases patients. Moreover, several clinical phenotypes of pemphigus may be associated with these antibodies. In this review we analyze clinicopathological, immunologic and outcome features of anti-Dsc autoimmune blistering diseases patients, to improve their diagnosis and management. We conducted a systematic search of PubMed and Embase (1990-present) for studies reporting cases of autoimmune blistering diseases with anti-Dsc antibodies. We classified the selected patients as patients with exclusively anti-Dsc autoantibodies, and patients with anti-Dsc and other autoantibodies. Of 93 cases with anti-Dsc autoantibodies included, 38 (41%) had exclusively these antibodies. Only 18% of patients presented with the typical clinicopathological phenotype of pemphigus vulgaris or pemphigus foliaceous. Mucosal involvement was seen in approximately half of the patients. Up to 18% of cases were associated with neoplasms. Acantholysis was described in 54% of cases with histopathological information. Treatments and outcomes vary in the different clinical phenotypes. The presence of anti-Dsc antibodies must be suspected mainly in those patients with either atypical pemphigus, in special with clinical pustules, or in cases showing intraepithelial or dermal neutrophilic/eosinophilic infiltrate on histological examination and dual pattern by direct immunofluorescence examination.
Highlights
Autoimmune blistering diseases (AIBD) encompass a group of disorders characterized clinically by the development of cutaneous and/or mucosal blisters and erosions
Anti-Dsc antibodies have mostly been involved in atypical pemphigus such as pemphigus herpetiformis (PH) and pemphigus vegetans (PVeg) [7]
We considered all patients with annular plaques with or without vesicles/pustules/ blisters as PH, and IgA pemphigus was considered as subcorneal pustular dermatosis (SPD) or intraepidermal neutrophilic dermatosis (IEND) based on the clinicopathological description from the author
Summary
Autoimmune blistering diseases (AIBD) encompass a group of disorders characterized clinically by the development of cutaneous and/or mucosal blisters and erosions. Pemphigus is a AIBD skin disease caused by the production of autoantibodies that target. Autoimmune-Blistering Diseases With Anti-Desmocollin Autoantibodies desmosomal adhesion molecules including desmoglein (Dsg) and desmocollin (Dsc), among others [1]. Dsg and Dsg are the major target antigens in pemphigus, but in the last 20 years, new relevant findings have demonstrated the role of non-Dsg autoantibodies in pemphigus cell-cell detachment [3, 4] even acting synergistically when Dsg autoantibodies are absent [5]. Dsc and 3 were among the top 10 most commonly recognized antigens in pemphigus vulgaris (PV) patients sera [6]. Anti-Dsc antibodies have mostly been involved in atypical pemphigus such as pemphigus herpetiformis (PH) and pemphigus vegetans (PVeg) [7]
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