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.
References
Kridin K, Patel PM, Jones VA, Cordova A, Amber KT. IgA pemphigus: A systematic review. J Am Acad Dermatol. 2020;82(6):1386-92. https://doi.org/10.1016/j.jaad.2019.11.059
Tsuruta D, Ishii N, Hamada T, Ohyama B, Fukuda S, Koga H, et al. IgA pemphigus. Clin Dermatol. 2011;29(4):437-42. https://doi.org/10.1016/j.clindermatol.2011.01.014
Męcińska-Jundziłł K, Hashimoto T, Kowalewski C, Woźniak K, Ishii N, Czajkowski R. Discre pancies among clinical, histological and im munological findings in IgA pemphigus: a case report and literature survey. Postepy Dermatol Alergol. 2016;33(6):480-4. https://doi.org/10.5114/pdia.2016.62486
Nishikawa T, Hashimoto T. Dermatoses with intraepidermal IgA deposits. Clin Dermatol. 2000;18(3):315-8. https://doi.org/10.1016/s0738-081x(99)00122-4
Di Milia M, Dickinson C, Candiz M, Forero O, Ri ganti J, Torre A, et al. Guías de Manejo de los Pén figos 2020. Sociedad Argentina de Dermatología; 2020. Disponible en: https://bit.ly/3Hw0nPf
Hodak E, David M, Ingber A, Rotem A, Hazaz B, Shamai-Lubovitz O, et al. The clinical and histo pathological spectrum of IgA-pemphigus--report of two cases. Clin Exp Dermatol. 1990;15(6):433-7. https://doi.org/10.1111/j.1365-2230.1990.tb02138.x
Bosio Bonet M, Bonetto V, Juárez-Villanueva L, Va lente E, Ruiz A, Kurpis M. Pénfigo por IgA variante dermatosis neutrofílica intraepidérmica. Med Cutan Iber Lat Am. 2017;45(2):165-8.
Schauer F, Kern JS, Kiritsi D. Systemic retinoids for treatment of recalcitrant IgA pemphigus. Orphanet J Rare Dis. 2018;13(1):163. https://doi.org/10.1186/s13023-018-0899-y
Moreno AC, Santi CG, Gabbi TV, Aoki V, Hashimoto T, Maruta CW. IgA pemphigus: case series with emphasis on therapeutic response. J Am Acad Der matol. 2014;70(1):200-1. https://doi.org/10.1016/j.jaad.2013.09.037
Gruss C, Zillikens D, Hashimoto T, Amagai M, Kroiss M, Vogt T, et al. Rapid response of IgA pem phigus of subcorneal pustular dermatosis type to treatment with isotretinoin. J Am Acad Dermatol. 2000;43(5 Pt 2):923-6. https://doi.org/10.1067/mjd.2000.104002
Howell SM, Bessinger GT, Altman CE, Belnap CM. Rapid response of IgA pemphigus of the subcor neal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil. J Am Acad Dermatol. 2005;53(3):541-3. https://doi.org/10.1016/j.jaad.2005.02.051
How to Cite
Downloads
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2023 Revista de la Asociación Colombiana de Dermatología y Cirugía Dermatológica
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
| Article metrics | |
|---|---|
| Abstract views | |
| Galley vies | |
| PDF Views | |
| HTML views | |
| Other views | |
