Bronchiolitis Obliterans With Anti-Epiplakin Antibodies in a Boy With Paraneoplastic Pemphigus

Author:

Rivas-Calderon Marian1,Yamazaki-Nakashimada Marco A.2,Orozco-Covarrubias Luz1,Durán-McKinster Carola1,Pacheco-Tovar Deyanira3,Ávalos-Díaz Esperanza3,Sáez-de-Ocariz Marimar1

Affiliation:

1. Dermatology Department

2. Immunology Department, National Institute of Pediatrics, Mexico City, Mexico

3. Department of Immunology, Unidad Académica de Ciencias Biológicas, Universidad Autónoma de Zacatecas, Mexico

Abstract

Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by a recalcitrant and severe mucositis, and polymorphic cutaneous lesions, associated with benign and malignant neoplasms. Paraneoplastic pemphigus is caused by production of autoantibodies against various epidermal proteins involved in cell adhesion. Bronchiolitis obliterans (BO) is one of the leading causes of mortality in these patients. Recent advances have associated the presence of anti-epiplakin antibodies with the development of BO in adult patients. Here we describe the first pediatric patient in whom the association of anti-epiplakin antibodies and BO have been reported so far.

Publisher

American Academy of Pediatrics (AAP)

Subject

Pediatrics, Perinatology and Child Health

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