Autoantibodies in Senear-usher Syndrome: Cross-reactivity or Multiple Autoimmunity?

Overview

Journal Autoimmune Dis

Publisher Wiley

Date 2013 Jan 16

PMID 23320149

Citations 7

Authors

Maria Elena Perez-Perez

Esperanza Avalos-Diaz

Rafael Herrera-Esparza

Affiliations

Soon will be listed here.

Abstract

Senear-Usher syndrome or pemphigus erythematosus is a pathology that overlaps clinically and serologically with pemphigus foliaceus and lupus erythematosus. Skin biopsies of patients with pemphigus erythematosus reveal acantholysis and deposits of immunoglobulins in desmosomes, and they are positive in the lupus band test. In the present paper, we determined whether the autoantibodies associated with pemphigus erythematosus targeted a single antigen or multiple antigens as a result of the stimulation of independent B cell clones. Our present paper demonstrates that patients with pemphigus erythematosus produce both antiepithelial antibodies specific for desmoglein 1 and 3 and antinuclear antibodies specific for Ro, La, Sm, and double-stranded DNA antigens. After eluting specific anti-epithelial or anti-nuclear antibodies, which were recovered and tested using double-fluorescence assays, a lack of cross-reactivity was demonstrated between desmosomes and nuclear and cytoplasmic lupus antigens. This result suggests that autoantibodies in pemphigus erythematosus are directed against different antigens and that these autoantibodies are produced by independent clones. Given these clinical and serological data, we suggest that pemphigus erythematosus behaves as a multiple autoimmune disease.

Citing Articles

Senear-Usher Syndrome or Coexistence of SLE with Pemphigus Vulgaris-A Case Report with Literature Review.

Kutwin M, Kadziela M, Stein T, Kraska-Gacka M, Wozniacka A, Zebrowska A J Clin Med. 2025; 14(2).

PMID: 39860415 PMC: 11765791. DOI: 10.3390/jcm14020409.

Senear-Usher syndrome in a 5-year-old girl.

Arasiewicz H, Czuwara J, Dec M, Lesniak-Jakubiec L Postepy Dermatol Alergol. 2024; 41(2):242-244.

PMID: 38784933 PMC: 11110225. DOI: 10.5114/ada.2024.139233.

A Recalcitrant Case of Senear-Usher Syndrome Treated With Rituximab.

de Vries J, Moody P, Ojha A, Grise A, Sami N Cureus. 2023; 15(10):e46910.

PMID: 37954755 PMC: 10639030. DOI: 10.7759/cureus.46910.

Autoimmune Pemphigus: Latest Advances and Emerging Therapies.

Lim Y, Bohelay G, Hanakawa S, Musette P, Janela B Front Mol Biosci. 2022; 8:808536.

PMID: 35187073 PMC: 8855930. DOI: 10.3389/fmolb.2021.808536.

Multiple autoimmunity and epitope spreading in monozygotic twins.

Avalos-Diaz E, Perez-Perez E, Granados J, Pacheco-Tovar D, Bollain-Y-Goytia-de-la-Rosa J, Herrera-Esparza R J Transl Autoimmun. 2021; 4:100132.

PMID: 34816112 PMC: 8593657. DOI: 10.1016/j.jtauto.2021.100132.

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