Limits...
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

Antigenic digestion of cow nose using enzymes. (a), trypsin digestion abrogated the antiepithelial antibody (AEA) titers. (b), nuclease (RNAse/DNAse) digestion abrogated the antinuclear antibody (ANA) titer, as demonstrated by indirect immunofluorescence.
© Copyright Policy
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC3539423&req=5

fig2: Antigenic digestion of cow nose using enzymes. (a), trypsin digestion abrogated the antiepithelial antibody (AEA) titers. (b), nuclease (RNAse/DNAse) digestion abrogated the antinuclear antibody (ANA) titer, as demonstrated by indirect immunofluorescence.

Mentions: The antinuclear reactivity of pemphigus erythematosus patient sera was abrogated in HEp-2 cells digested with nucleases. The nuclear fluorescence disappeared in the cow nose cells, but fluorescence was still present in the intercellular spaces. In contrast, the intercellular reactivity disappeared but the nuclear fluorescence remained positive in cow nose treated with trypsin. Finally, in a set of cow nose tissue samples double digested with trypsin and nucleases all fluorescence was abrogated. Control experiments using high ANA-titer lupus serum were negative for cow nose digested with nucleases. In addition, the pemphigus vulgaris serum with a high AEA titer was negative with trypsin-digested cow nose. The results of this experiment demonstrate the ability of the digestion procedure to eliminate these antigens and that one serum sample can have two different specificities: one that is antiepithelial and another that is antinuclear (Figure 2).


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

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

Antigenic digestion of cow nose using enzymes. (a), trypsin digestion abrogated the antiepithelial antibody (AEA) titers. (b), nuclease (RNAse/DNAse) digestion abrogated the antinuclear antibody (ANA) titer, as demonstrated by indirect immunofluorescence.
© Copyright Policy
Related In: Results  -  Collection

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

fig2: Antigenic digestion of cow nose using enzymes. (a), trypsin digestion abrogated the antiepithelial antibody (AEA) titers. (b), nuclease (RNAse/DNAse) digestion abrogated the antinuclear antibody (ANA) titer, as demonstrated by indirect immunofluorescence.
Mentions: The antinuclear reactivity of pemphigus erythematosus patient sera was abrogated in HEp-2 cells digested with nucleases. The nuclear fluorescence disappeared in the cow nose cells, but fluorescence was still present in the intercellular spaces. In contrast, the intercellular reactivity disappeared but the nuclear fluorescence remained positive in cow nose treated with trypsin. Finally, in a set of cow nose tissue samples double digested with trypsin and nucleases all fluorescence was abrogated. Control experiments using high ANA-titer lupus serum were negative for cow nose digested with nucleases. In addition, the pemphigus vulgaris serum with a high AEA titer was negative with trypsin-digested cow nose. The results of this experiment demonstrate the ability of the digestion procedure to eliminate these antigens and that one serum sample can have two different specificities: one that is antiepithelial and another that is antinuclear (Figure 2).

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