IgA pemphigus
DOI:
https://doi.org/10.29176/2590843X.1608Keywords:
Bullous disorder, IgA, PemphigusAbstract
IgA pemphigus is a rare, chronic bullous disease characterized by the presence of IgA autoantibodies against desmosomes. Two main variants are recognized: the subcorneal pustular dermatosis (SCD) type and intraepidermal neutrophilic dermatosis (NIE). The diagnosis is based on the clinical features and is confirmed by histology and the presence of IgA deposits between the keratinocytes. Dapsone is the
first-line treatment. In this report, we presented a patient with the intraepidermal neutrophilic dermatosis variant who initially responded to oral corticosteroids and dapsone. The purpose of this report is to briefly review the most important features of this rare entity.
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