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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

Double fluorescence assay using cow nose tissue. Cellular junctions were tagged in green (FITC) with labelled antiepithelial antibodies, and nuclei were tagged in red with labelled antinuclear antibodies (Texas red).
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fig3: Double fluorescence assay using cow nose tissue. Cellular junctions were tagged in green (FITC) with labelled antiepithelial antibodies, and nuclei were tagged in red with labelled antinuclear antibodies (Texas red).

Mentions: The affinity-purified antiepithelial antibodies tested on undigested cow nose and tagged in green were visualised in the intercellular spaces, producing a honeycomb fluorescence pattern characteristic of pemphigus. The affinity-purified antinuclear antibodies tagged in red were bound along the nuclei of keratinocytes. The merged image in Figure 3 shows fluorescence in two colours.


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

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

Double fluorescence assay using cow nose tissue. Cellular junctions were tagged in green (FITC) with labelled antiepithelial antibodies, and nuclei were tagged in red with labelled antinuclear antibodies (Texas red).
© Copyright Policy
Related In: Results  -  Collection

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

fig3: Double fluorescence assay using cow nose tissue. Cellular junctions were tagged in green (FITC) with labelled antiepithelial antibodies, and nuclei were tagged in red with labelled antinuclear antibodies (Texas red).
Mentions: The affinity-purified antiepithelial antibodies tested on undigested cow nose and tagged in green were visualised in the intercellular spaces, producing a honeycomb fluorescence pattern characteristic of pemphigus. The affinity-purified antinuclear antibodies tagged in red were bound along the nuclei of keratinocytes. The merged image in Figure 3 shows fluorescence in two colours.

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