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Rafael Herrera-esparza - One of the best experts on this subject based on the ideXlab platform.
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Research Article Autoantibodies in Senear-Usher Syndrome: Cross-Reactivity or Multiple Autoimmunity?
2015Co-Authors: Rafael Herrera-esparzaAbstract:Copyright © 2012 Marı́a Elena Pérez-Pérez et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. 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. 1. Backgroun
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Autoantibodies in senear-usher syndrome: cross-reactivity or multiple autoimmunity?
Autoimmune Diseases, 2012Co-Authors: María Elena Pérez-pérez, Esperanza Avalos-díaz, Rafael Herrera-esparzaAbstract: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.
María Elena Pérez-pérez - One of the best experts on this subject based on the ideXlab platform.
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Autoantibodies in senear-usher syndrome: cross-reactivity or multiple autoimmunity?
Autoimmune Diseases, 2012Co-Authors: María Elena Pérez-pérez, Esperanza Avalos-díaz, Rafael Herrera-esparzaAbstract: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.
Lawrence S Chan - One of the best experts on this subject based on the ideXlab platform.
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Unusually extensive scalp ulcerations manifested in Pemphigus Erythematosus.
Dermatology Online Journal, 2018Co-Authors: Neha Chandan, Eden Lake, Lawrence S ChanAbstract:Author(s): Chandan, Neha; Lake, Eden P; Chan, Lawrence S | Abstract: Senear-Usher Syndrome, or Pemphigus Erythematosus, is an autoimmune skin blistering disorder with an overlapping clinical presentation of Pemphigus foliaceus and lupus Erythematosus. Lesions typically involve the scalp, face, and upper chest or back. This case study focuses on a patient who presentedwith progressive scalp ulcers, hyperpigmentation, and eroded plaques with overlying hemorrhagic crust. Pemphigus Erythematosus was diagnosedwith direct immunofluorescence, demonstrating immunoglobulin G and complement deposition both intercellularly and at the dermoepidermal junction. The patient is continuing treatment with systemicsteroids and steroid-sparing immunosuppressants.
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Unusually extensive scalp ulcerations manifested in Pemphigus Erythematosus.
Dermatology online journal, 2018Co-Authors: Neha Chandan, Eden Lake, Lawrence S ChanAbstract:Senear-Usher Syndrome, or Pemphigus Erythematosus, is an autoimmune skin blistering disorder with an overlapping clinical presentation of Pemphigus foliaceus and lupus Erythematosus. Lesions typically involve the scalp, face, and upper chest or back. This case study focuses on a patient who presentedwith progressive scalp ulcers, hyperpigmentation, and eroded plaques with overlying hemorrhagic crust. Pemphigus Erythematosus was diagnosedwith direct immunofluorescence, demonstrating immunoglobulin G and complement deposition both intercellularly and at the dermoepidermal junction. The patient is continuing treatment with systemicsteroids and steroid-sparing immunosuppressants.
Esperanza Avalos-díaz - One of the best experts on this subject based on the ideXlab platform.
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Autoantibodies in senear-usher syndrome: cross-reactivity or multiple autoimmunity?
Autoimmune Diseases, 2012Co-Authors: María Elena Pérez-pérez, Esperanza Avalos-díaz, Rafael Herrera-esparzaAbstract: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.
A. Lanza - One of the best experts on this subject based on the ideXlab platform.
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Mucocutaneous Pemphigus vulgaris carrying high-titre antidesmoglein 1 antibodies with skin lesions resembling Pemphigus Erythematosus
Clinical and Experimental Dermatology, 2007Co-Authors: N. Cirillo, R. Santoro, M. Lanza, P. Annese, F. Gombos, A. LanzaAbstract:Patients with Pemphigus vulgaris (PV) who have both antidesmoglein (Dsg)1 and anti-Dsg3 antibodies usually develop flaccid blisters on skin and mucous membranes. We report a case of PV with crusting skin lesions resembling Pemphigus Erythematosus, the localized variant of Pemphigus foliaceus (PF)
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Mucocutaneous Pemphigus vulgaris carrying high-titre antidesmoglein 1 antibodies with skin lesions resembling Pemphigus Erythematosus.
Clinical and experimental dermatology, 2007Co-Authors: N. Cirillo, R. Santoro, M. Lanza, P. Annese, F. Gombos, A. LanzaAbstract:Summary Patients with Pemphigus vulgaris (PV) who have both antidesmoglein (Dsg)1 and anti-Dsg3 antibodies usually develop flaccid blisters on skin and mucous membranes. We report a case of PV with crusting skin lesions resembling Pemphigus Erythematosus, the localized variant of Pemphigus foliaceus (PF). Notably, the patient had high titres of anti-Dsg1 IgG, as assessed by ELISA. We then established an in vitro model of Pemphigus, and found that patient's serum was able to induce suprabasilar acantholysis in mouse skin culture. However, epidermal splitting also occurred within the granular layer, suggesting that the pathogenic potential of such a high-titre anti-Dsg1 serum was intermediate between PV and PF. Thus, the levels of anti-Dsg1 antibodies could play a role in determining the clinical phenotype of Pemphigus.