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Autoantibodies in senear-usher syndrome: cross-reactivity or multiple autoimmunity?

Pérez-Pérez ME, Avalos-Díaz E, Herrera-Esparza R - Autoimmune Dis (2012)

Bottom Line: 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.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Immunology, Universidad Autónoma de Zacatecas, Chepinque 306, Colonia Lomas de la Soledad, 98040 Zacatecas, ZAC, Mexico.

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.

No MeSH data available.


Related in: MedlinePlus

Antibody followup in three patients with pemphigus erythematosus (average of antibody titer).
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fig5: Antibody followup in three patients with pemphigus erythematosus (average of antibody titer).

Mentions: To define the molecular evolution of the immune response, we tracked the immune specificity of serum from three patients over ten years. The results of this analysis yielded the conclusion that the desmoglein complex (Dsg 1) is the initial autoantibody target because antiepithelial antibodies appeared several months or years before the antinuclear response. Furthermore, the immunodominant epitope targeted the desmosome complex, as demonstrated by the antiepithelial antibody titers being higher (Figure 5). Based on this analysis, we can conclude that the desmosome complex is the initial antigenic target, and epitope spreading occurs subsequently, leading to the production of autoantibodies against ribonucleoproteins.


Autoantibodies in senear-usher syndrome: cross-reactivity or multiple autoimmunity?

Pérez-Pérez ME, Avalos-Díaz E, Herrera-Esparza R - Autoimmune Dis (2012)

Antibody followup in three patients with pemphigus erythematosus (average of antibody titer).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3539423&req=5

fig5: Antibody followup in three patients with pemphigus erythematosus (average of antibody titer).
Mentions: To define the molecular evolution of the immune response, we tracked the immune specificity of serum from three patients over ten years. The results of this analysis yielded the conclusion that the desmoglein complex (Dsg 1) is the initial autoantibody target because antiepithelial antibodies appeared several months or years before the antinuclear response. Furthermore, the immunodominant epitope targeted the desmosome complex, as demonstrated by the antiepithelial antibody titers being higher (Figure 5). Based on this analysis, we can conclude that the desmosome complex is the initial antigenic target, and epitope spreading occurs subsequently, leading to the production of autoantibodies against ribonucleoproteins.

Bottom Line: 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.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Immunology, Universidad Autónoma de Zacatecas, Chepinque 306, Colonia Lomas de la Soledad, 98040 Zacatecas, ZAC, Mexico.

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.

No MeSH data available.


Related in: MedlinePlus