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Therapeutic Approach

Intercellular IgA dermatosis (IAD), which is also called as IgA pemphigus, is a rare autoimmune bullous disease, characterized by tissue-bound and circulating IgA autoantibodies against epidermal keratinocyte cell surfaces. IAD is divided into two major subtypes, subcorneal pustular dermatosis (SPD) type and intraepidermal neutrophilic IgA dermatosis (IEN) type, with minor subtypes of IgA pemphigus vegetans, IgA pemphigus foliaceus, IgA pemphigus vulgaris, and undetermined type. Therapies consist of both anti-inflammatory and immunosuppressive medications. However, consensus for the treatments for IAD has not been obtained. See Table 43-1.

Table 43-1Intercellular IgA Dermatosis/IgA Pemphigus Treatment Table

Levels of evidence are based on the Journal of the American Academy of Dermatology guidelines: level IA evidence includes evidence from meta-analysis of randomized controlled trials; level IB ...

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