What is superficial IgA pemphigus?


Isolated pustule and annular lesions demonstrating scale-crust superficial pustules in a patient with IgA pemphigus. In the past, this would have been considered to be subcorneal pustular dermatosis.
Fig. 11.3 Isolated pustule and annular lesions demonstrating scale-crust superficial pustules in a patient with IgA pemphigus. In the past, this would have been considered to be subcorneal pustular dermatosis.
Recently, it has been demonstrated that some cases of what were formerly classified as subcorneal pustular dermatosis may demonstrate intraepidermal IgA between the keratinocytes. Most authorities feel that cases with intraepidermal IgA on direct immunofluorescence should be reclassified as superficial IgA pemphigus (Fig. 11-3). It has been demonstrated that the IgA autoantibodies are directed against desmocollin 1 and possibly desmocollins 2 and 3, which are molecules that are important for normal adhesion between keratinocytes.

Düker I, Schaller J, Rose C, et al: Subcorneal pustular dermatosis-type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3, Arch Dermatol 145:1159–1162, 2009.