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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

(a) Indirect immunofluorescence in cow nose tissue showing a honeycomb fluorescence pattern along intercellular junctions of keratinocytes that is characteristic of pemphigus and a nuclear fluorescence pattern for ANA. (b) The same serum sample was tested in HEp-2 cells, resulting in a homogeneous nuclear and nucleolar pattern. (c) Graph of individual titers of each serum sample.
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fig1: (a) Indirect immunofluorescence in cow nose tissue showing a honeycomb fluorescence pattern along intercellular junctions of keratinocytes that is characteristic of pemphigus and a nuclear fluorescence pattern for ANA. (b) The same serum sample was tested in HEp-2 cells, resulting in a homogeneous nuclear and nucleolar pattern. (c) Graph of individual titers of each serum sample.

Mentions: All of the sera were positive for pemphigus antiepithelial antibodies at variable titers, and the antibodies in eight sera reacted with desmogleins (Dsg). With regard to lupus specificity, all of the sera tested positive for antinuclear antibodies at titers above 1 : 160 on HEp-2 cells. The dominant fluorescence pattern was fine speckles (in six sera). One serum sample had a homogeneous nucleolar pattern, and the remaining sera sample exhibited diffuse nuclear fluorescence (Figure 1).


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

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

(a) Indirect immunofluorescence in cow nose tissue showing a honeycomb fluorescence pattern along intercellular junctions of keratinocytes that is characteristic of pemphigus and a nuclear fluorescence pattern for ANA. (b) The same serum sample was tested in HEp-2 cells, resulting in a homogeneous nuclear and nucleolar pattern. (c) Graph of individual titers of each serum sample.
© Copyright Policy
Related In: Results  -  Collection

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

fig1: (a) Indirect immunofluorescence in cow nose tissue showing a honeycomb fluorescence pattern along intercellular junctions of keratinocytes that is characteristic of pemphigus and a nuclear fluorescence pattern for ANA. (b) The same serum sample was tested in HEp-2 cells, resulting in a homogeneous nuclear and nucleolar pattern. (c) Graph of individual titers of each serum sample.
Mentions: All of the sera were positive for pemphigus antiepithelial antibodies at variable titers, and the antibodies in eight sera reacted with desmogleins (Dsg). With regard to lupus specificity, all of the sera tested positive for antinuclear antibodies at titers above 1 : 160 on HEp-2 cells. The dominant fluorescence pattern was fine speckles (in six sera). One serum sample had a homogeneous nucleolar pattern, and the remaining sera sample exhibited diffuse nuclear fluorescence (Figure 1).

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