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.
Patients with IGAP may present with:
- Crusting and superficial pustules (Subcorneal pustular dermatosis-like)
- IgA localized to the upper epidermis of DIF
- Antibodies to desmocollin 1
- Grouped, tense vesicobullae and pustules
- IgA deposition throughout the epidermis
- Antibodies to desmoglein 3, the pemphigus vulgaris antigen
In addition to skin biopsy and DIF, various cultures should be obtained to exclude infection.
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].
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.
Homepage | FAQs | Use of Images | Contact Dr. White
It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.