Autoantibodies in Senear-Usher Syndrome: Cross-Reactivity or Multiple Autoimmunity?

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.

Autoantibodies to full body vascular cell junctions colocalize with MYZAP, ARVCF, desmoplakins I and II and p0071 in endemic pemphigus in Colombia, South America

“…We also blocked any nonspecific immune responses that can be seen when IIF or DIF are performed on vascular tissues. El Bagre‐EPF has autoantibodies to multiple organs as likely occurs in Senear‐Usher syndrome that may require further studies investigating cross‐reactivity or multiple autoimmunity …”

Desmosomes in acquired disease

“…Dsgs 1 and 3, the primary targets in pemphigus vulgaris, are also targets in pemphigus erythematosus, a condition with pathology that overlaps clinically and serologically with pemphigus foliaceus and lupus erythematosus (Oktarina, et al, 2012, Perez-Perez, et al, 2012). In this autoimmune disease, IgG deposition is mainly found in the granular layer of the epidermis, suggesting that Dsg1 is the primary pathogenic autoantigen (Amerian and Ahmed, 1985).…”

“…Thus, the condition has recently been classified as a variant of pemphigus foliaceus (Steffen and Thomas, 2003). Additionally, antinuclear antibodies are also present in most patients with pemphigus erythematosus (Kneisel and Hertl, 2011a, Perez-Perez, et al, 2012, Steffen and Thomas, 2003). In patients with Grover’s disease, an itchy acantholytic dermatosis, staining for Dsg1 and/or Dsg3 was shown to be decreased..…”

An unusual case of pemphigus erythematosus

“…[11,12] AntiDsg1 antibodies deposite along dermo-epidermal junction mimicking in ANA negative patients. [8,13] As none of the direct immunofluorescence-positive patients was anti-Dsgnegative, [12] in this case we did not perform ELISA because we already found IgG and C3 from direct immunofluorescence staining.…”

“…[7] In our patient, we observed prominent papules, plaques, and a few pustules accompanied by pruritus, that were different from the vesiculobullous lesion in other pemphigus erythematosus patients. Common distribution of pemphigus erythematosus spread to the scalp, malar area, [8] and trunk, [5] but mainly in seborrheic area. [2] This distribution of skin lesions were found in our patient.…”