IgA PEMPHIGUS

By Gary M. White, MD


IgA pemphigus (IGAP), also known as Intraepidermal Neutrophilic IgA Dermatosis, is an autoimmune bullous dermatosis caused by IgA binding to the epidermis.

Clinical

Patients with IGAP may present with:

Workup

In addition to skin biopsy and DIF, various cultures should be obtained to exclude infection.

Treatment

Dapsone either alone or in combination with prednisone is usually effective. Colchicine 1.5 mg/day as monotherapy has also been used [JAAD 1999;40;91]. Oral retinoids (e.g. acitretin and isotretinoin) as well as mycophenolate mofetil and adalimumab have been reported effective. Azathioprine has been reported as not effective. IVIG has been used [JEADV 2016;June;30;1055].

References

This image from N Engl J Med 2012; 366:e31 shows blisters half-filled with pus and the rest clear fluid in a patient with subcorneal pustular dermatosis/IgA pemphigus overlap.

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