Severe pemphigus erythematosus associated with skin failure successfully treated with rituximab
Case report
DOI:
https://doi.org/10.29176/2590843X.1755Keywords:
Desmoglein 1, Pemphigus, RituximabAbstract
Pemphigus erythematosus is an uncommon intraepithelial autoimmune blistering disease mediated by autoantibodies targeting desmoglein 1. It has a broad clinical spectrum, in severe cases can result in multi-organ compromise. To date, there is no established treatment for severe cases of PE. We describe a case of a previously healthy patient with a 4 month clinical course of generalized painful erythematous desquamative plaques and blisters, associated with a 10 kg weight loss. Clinical and histopathological findings were consistent with PE. An adjusted Rituximab protocol was used, which is a well known effective treatment for Pemphigus vulgaris, nevertheless there aren't many reports. Our patient achieved disease remission within 20 days.
Author Biographies
Bruny Carolina Llamas Castellanos, Fundación Santa Fe
Asistente de Investigación, Hospital Universitario Fundación Santa Fe de Bogotá, Bogotá, Colombia. Universidad del Rosario, Bogotá, Colombia
Iván Pérez-Háded, Hospital Universitario Fundación Santa Fe de Bogotá, Bogotá, Colombia
Departamento de Dermatología, Hospital Universitario Fundación Santa Fe de Bogotá, Bogotá, Colombia
Laura C. García-Medina, Universidad de los Andes, Bogotá, Colombia
Universidad de los Andes, Bogotá, Colombia
Juliana Criales-Laguado, Universidad de los Andes, Bogotá, Colombia
Universidad de los Andes, Bogotá, Colombia
References
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Almugairen N, Hospital V, Bedane C, Duvert-Lehembre S, Picard D, Tronquoy A et al. Assessment of the rate of long-term complete remission off therapy in patients with pemphigus treated with different regimens including medium- and high-dose corticosteroids. Journal of the American Academy of Dermatology. 2013;69(4):583-588. https://doi.org/10.1016/j.jaad.2013.05.016
Joly P, Maho-Vaillant M, Prost-Squarcioni C, Hebert V, Houivet E, Calbo S et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomized trial. The Lancet. 2017;389(10083):2031-2040. https://doi.org/10.1016/S0140-6736(17)30070-3
Wang H, Liu C, Li Y, Huang Y. Efficacy of Rituximab for Pemphigus: A Systematic Review and Meta-analysis of Different Regimens. Acta Dermato Venereologica. 2015;95(8):928-932. https://doi.org/10.2340/00015555-2116
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Copyright (c) 2024 Bruny Carolina Llamas Castellanos, Iván Pérez-Háded, Laura C. García-Medina, Juliana Criales-Laguado
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
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