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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

Multiple molecular reactivity of pemphigus erythematosus patient sera to desmosome and ribonucleoprotein components. This result may indicate intermolecular antigen spreading.
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fig4: Multiple molecular reactivity of pemphigus erythematosus patient sera to desmosome and ribonucleoprotein components. This result may indicate intermolecular antigen spreading.

Mentions: Similarly, the affinity-purified antibodies eluted from cow nose digested with trypsin displayed anti-Ro, anti-La, anti-Sm/RNP and anti-DNA activities but not anti-Dsg1, or -Dsg3 activity. This experiment confirmed the hypothesis that sera from pemphigus erythematosus patients possess two distinct families of autoantibodies, one corresponding to pemphigus and directed against desmosome proteins and the other directed against nucleocytoplasmic lupus antigens, such as ribonucleoproteins or deoxyribonucleoproteins (Figure 4).


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

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

Multiple molecular reactivity of pemphigus erythematosus patient sera to desmosome and ribonucleoprotein components. This result may indicate intermolecular antigen spreading.
© Copyright Policy
Related In: Results  -  Collection

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

fig4: Multiple molecular reactivity of pemphigus erythematosus patient sera to desmosome and ribonucleoprotein components. This result may indicate intermolecular antigen spreading.
Mentions: Similarly, the affinity-purified antibodies eluted from cow nose digested with trypsin displayed anti-Ro, anti-La, anti-Sm/RNP and anti-DNA activities but not anti-Dsg1, or -Dsg3 activity. This experiment confirmed the hypothesis that sera from pemphigus erythematosus patients possess two distinct families of autoantibodies, one corresponding to pemphigus and directed against desmosome proteins and the other directed against nucleocytoplasmic lupus antigens, such as ribonucleoproteins or deoxyribonucleoproteins (Figure 4).

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