Autoimmune Skin Disease

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Luis A Diaz - One of the best experts on this subject based on the ideXlab platform.

  • eosinophils mediate tissue injury in the Autoimmune Skin Disease bullous pemphigoid
    Journal of Investigative Dermatology, 2017
    Co-Authors: Lan Lin, Janet A Fairley, Bin Jin Hwang, Donna A Culton, Susan Burette, Beverly H Koller, Kelly A Messingham, James J Lee, Russell P Hall, Luis A Diaz
    Abstract:

    Eosinophils are typically associated with unique inflammatory settings, including allergic inflammation and helminth infections. However, new information suggests that eosinophils contribute more broadly to inflammatory responses and participate in local immune regulation and the tissue remodeling/repair events linked with a variety of Diseases. Eosinophilic infiltration has long been a histologic hallmark of bullous pemphigoid (BP), a subepidermal Autoimmune blistering Disease characterized by autoantibodies directed against basement membrane protein BP180. However, the exact role of eosinophils in Disease pathogenesis remains largely unknown. We show here that eosinophils are necessary for IgE autoantibody-mediated BP blister formation in a humanized IgE receptor mouse model of BP. Disease severity is IgE dose dependent and correlates with the degree of eosinophil infiltration in the Skin. Furthermore, IgE autoantibodies fail to induce BP in eosinophil-deficient mice, confirming that eosinophils are required for IgE-mediated tissue injury. Thus, eosinophils provide the cellular link between IgE autoantibodies and Skin blistering in this murine model of BP. These findings suggest a role for eosinophils in Autoimmune Disease and have important implications for the treatment of BP and other antibody-mediated inflammatory and Autoimmune Diseases.

  • Non-infectious environmental antigens as a trigger for the initiation of an Autoimmune Skin Disease☆
    Autoimmunity reviews, 2016
    Co-Authors: Ye Qian, Donna A Culton, Joseph S. Jeong, Nicole Trupiano, Jesus G. Valenzuela, Luis A Diaz
    Abstract:

    Pemphigus represents a group of organ specific Autoimmune blistering disorders of the Skin mediated by pathogenic autoantibodies with well-defined antigenic targets. While most of these Diseases are sporadic, endemic forms of Disease do exist. The endemic form of pemphigus foliaceus (also known as fogo selvagem, FS) exhibits epidemiological features that suggest exposure to hematophagous insect bites are a possible precipitating factor of this Autoimmune Disease, and provides a unique opportunity to study how environmental factors contribute to Autoimmune Disease development. FS patients and healthy individuals from endemic regions show an autoreactive IgM response that starts in early childhood and becomes restricted to IgG4 autoantibodies in FS patients. In searching for triggering environmental antigens, we have found that IgG4 and IgE autoantibodies from FS patients cross-react with a salivary antigen from sand flies. The presence of these cross-reactive antibodies and antibody genetic analysis confirming that these antibodies evolve from the same naive B cells provides compelling evidence that this non-infectious environmental antigen could be the initial target of the autoantibody response in FS. Consequently, FS serves as an ideal model to study the impact of environmental antigens in the development of Autoimmune Disease.

  • involvement of the apoptotic mechanism in pemphigus foliaceus Autoimmune injury of the Skin
    Journal of Immunology, 2009
    Co-Authors: Ning Li, Minglang Zhao, Jinzhao Wang, Luis A Diaz
    Abstract:

    Pemphigus foliaceus (PF) is an organ-specific Autoimmune Skin Disease characterized by subcorneal epidermal cell detachment (acantholysis) and pathogenic autoantibodies against desmoglein 1. The mechanism responsible for pemphigus autoantibody-induced epidermal injury is not fully understood. In this study, we used the IgG passive transfer mouse model of PF to investigate the relevance of the apoptotic mechanism in pemphigus pathogenesis. TUNEL-positive epidermal cells and increased oligonucleosomes in the epidermal cytosolic fractions were detected in the Diseased mice. Time course study reveals that TUNEL-positive epidermal cells appear before intraepidermal blisters. Moreover, the proapoptotic factor Bax was up-regulated at the earlier time points (2 and 4 h), whereas the antiapoptotic factor Bcl-x L was down-regulated at the later time points (6, 8, and 20 h) post-PF IgG injection by Western blot analysis. The active forms of caspase-3 and -6 were detected at the later time period (6, 8, and 20 h). Administration of Ac-DEVD-cmk, a peptide-based caspase-3/7 inhibitor, protected mice from developing intraepidermal blisters and clinical Disease induced by PF IgG. The same protective effect was also observed using a broad-spectrum caspase inhibitor, Bok-D-fmk. Collectively, these findings show that biochemical events of apoptosis are provoked in the epidermis of mice injected with PF autoantibodies. Caspase activation may contribute to acantholytic blister formation in PF.

  • The Prevalence of Antibodies against Desmoglein 1 in Endemic Pemphigus Foliaceus in Brazil
    The New England journal of medicine, 2000
    Co-Authors: Simon Warren, Vandir Dos Santos, George J. Giudice, Mong Shang Lin, Raymond G. Hoffmann, Gunter Hans-filho, Evandro A. Rivitti, Luis A Diaz
    Abstract:

    Background Pemphigus foliaceus is an Autoimmune Skin Disease mediated by autoantibodies against desmoglein 1. The endemic form is thought to have an environmental cause. The Terena reservation of Limao Verde in Mato Grosso do Sul, Brazil, is a recently identified focus of the Disease, with a prevalence of 3.4 percent in the population. We tested the hypothesis that normal subjects living in an endemic area have antibodies against desmoglein 1. Methods We used an enzyme-linked immunosorbent assay to detect antibodies against desmoglein 1 in serum samples from 60 patients with endemic pemphigus foliaceus (fogo selvagem) who lived in Limao Verde or elsewhere in Brazil, 372 normal subjects (without pemphigus foliaceus) from Limao Verde and surrounding locations, and 126 normal subjects from the United States and Japan. Results Antibodies against desmoglein 1 were detected in 59 of the 60 patients with fogo selvagem (98 percent) but in only 3 of the 126 normal subjects from the United States and Japan (2 perce...

  • a critical role for neutrophil elastase in experimental bullous pemphigoid
    Journal of Clinical Investigation, 2000
    Co-Authors: Zhi Liu, George J. Giudice, Steven D Shapiro, Robert M, Xiaoye Zhou, Sally S Twining, Janet A Fairley, Luis A Diaz
    Abstract:

    Bullous pemphigoid (BP) is an Autoimmune Skin Disease characterized by subepidermal blisters and autoantibodies against 2 hemidesmosome-associated proteins, BP180 and BP230. The immunopathologic features of BP can be reproduced in mice by passive transfer of anti-BP180 antibodies. Lesion formation in this animal model depends upon complement activation and neutrophil recruitment. In the present study, we investigated the role of neutrophil elastase (NE) in antibody-induced blister formation in experimental BP. Abnormally high levels of caseinolytic activity, consistent with NE, were detected in extracts of lesional Skin and blister fluid of mice injected with anti-BP180 IgG. The pathogenic anti-BP180 IgG failed to induce subepidermal blistering in NE-null (NE‐/‐) mutant mice. NE ‐/‐ mice reconstituted with neutrophils from wild-type mice became susceptible to experimental BP. Wild-type mice given NE inhibitors (α1-proteinase inhibitor and Me-O-Suc-Ala-Ala-ProVal-CH2Cl), but not mice given cathepsin G/chymase inhibitors (α1-antichymotrypsin or Z-Gly-LeuPhe-CH2Cl), were resistant to the pathogenic activity of anti-BP180 antibodies. Incubation of murine Skin with NE induced BP-like epidermal-dermal detachment. Finally, NE cleaved BP180 in vitro and in vivo. These results implicate NE directly in the dermal-epidermal cleavage induced by anti-BP180 antibodies in the experimental BP model. J. Clin. Invest. 105:113‐123 (2000).

Hendri H. Pas - One of the best experts on this subject based on the ideXlab platform.

  • The IgG "Lupus-Band" Deposition Pattern of Pemphigus Erythematosus Association With the Desmoglein 1 Ectodomain as Revealed by 3 Cases
    Archives of dermatology, 2012
    Co-Authors: Dyah A. M. Oktarina, Gilles F. H. Diercks, Marcel F. Jonkman, Angelique Poot, Duco Kramer, Hendri H. Pas
    Abstract:

    Background Pemphigus foliaceus is an Autoimmune Skin Disease characterized by subcorneal blistering and IgG antibodies directed against desmoglein 1. In the Skin, these antibodies deposit intraepidermally. On rare occasions, an additional “lupus band” of granular depositions of IgG and complement is seen along the epidermal basement membrane zone. This combined pattern has been connected with a variant of pemphigus foliaceus named pemphigus erythematosus. Observations We describe 3 pemphigus foliaceus cases in which phototherapy was administered after a misdiagnosis of psoriasis. This treatment resulted in a flare of Skin lesions. Direct immunofluorescence of Skin biopsy specimens that were obtained several weeks later demonstrated intraepidermal and granular basement membrane zone depositions. The basement membrane zone depositions consisted of IgG, complement, and the ectodomain of desmoglein 1 and were located below the lamina densa. Conclusions High doses of UV light are likely to induce the cleaving of the desmoglein 1 ectodomain. In patients with pemphigus foliaceus, the circulating anti–desmoglein 1 antibodies precipitate this cleaved-off ectodomain along the basement membrane zone, resulting in a lupus band–like appearance. In pemphigus erythematosus, a similar mechanism may be active, which might explain the lupus-band phenomenon.

Dyah A. M. Oktarina - One of the best experts on this subject based on the ideXlab platform.

  • The IgG "Lupus-Band" Deposition Pattern of Pemphigus Erythematosus Association With the Desmoglein 1 Ectodomain as Revealed by 3 Cases
    Archives of dermatology, 2012
    Co-Authors: Dyah A. M. Oktarina, Gilles F. H. Diercks, Marcel F. Jonkman, Angelique Poot, Duco Kramer, Hendri H. Pas
    Abstract:

    Background Pemphigus foliaceus is an Autoimmune Skin Disease characterized by subcorneal blistering and IgG antibodies directed against desmoglein 1. In the Skin, these antibodies deposit intraepidermally. On rare occasions, an additional “lupus band” of granular depositions of IgG and complement is seen along the epidermal basement membrane zone. This combined pattern has been connected with a variant of pemphigus foliaceus named pemphigus erythematosus. Observations We describe 3 pemphigus foliaceus cases in which phototherapy was administered after a misdiagnosis of psoriasis. This treatment resulted in a flare of Skin lesions. Direct immunofluorescence of Skin biopsy specimens that were obtained several weeks later demonstrated intraepidermal and granular basement membrane zone depositions. The basement membrane zone depositions consisted of IgG, complement, and the ectodomain of desmoglein 1 and were located below the lamina densa. Conclusions High doses of UV light are likely to induce the cleaving of the desmoglein 1 ectodomain. In patients with pemphigus foliaceus, the circulating anti–desmoglein 1 antibodies precipitate this cleaved-off ectodomain along the basement membrane zone, resulting in a lupus band–like appearance. In pemphigus erythematosus, a similar mechanism may be active, which might explain the lupus-band phenomenon.

  • ONLINE FIRST OBSERVATION The IgG “Lupus-Band ” Deposition Pattern
    2012
    Co-Authors: Of Pemphigus Erythematosus, Gilles F. H. Diercks, Dyah A. M. Oktarina, Phd Angelique, M. Poot, Md Duco Kramer, Arch Dermatol
    Abstract:

    Background: Pemphigus foliaceus is an Autoimmune Skin Disease characterized by subcorneal blistering and IgG antibodies directed against desmoglein 1. In the Skin, these antibodies deposit intraepidermally. On rare occasions, an additional “lupus band ” of granular depositions of IgG and complement is seen along the epidermal basement membrane zone. This combined pattern has been connected with a variant of pemphigus foliaceus named pemphigus erythematosus. Observations: We describe 3 pemphigus foliaceus cases in which phototherapy was administered after a misdiagnosis of psoriasis. This treatment resulted in a flare of Skin lesions. Direct immunofluorescence of Skin biopsy specimens that were obtained several weeks later demonstrated intraepidermal and granular basement membrane zone depositions. The basement membrane zone depositions consisted of IgG, complement, and the ectodomain of desmoglein 1 and were located below the lamina densa. Conclusions: High doses of UV light are likely to induce the cleaving of the desmoglein 1 ectodomain. In patients with pemphigus foliaceus, the circulating anti– desmoglein 1 antibodies precipitate this cleaved-off ectodomain along the basement membrane zone, resulting in a lupus band–like appearance. In pemphigus erythematosus, a similar mechanism may be active, which might explain the lupus-band phenomenon

  • Association With the Desmoglein 1 Ectodomain as Revealed by 3 Cases
    2012
    Co-Authors: Of Pemphigus Erythematosus, Gilles F. H. Diercks, Dyah A. M. Oktarina, Phd Angelique, M. Poot, Md Duco Kramer, Phd Marcel, F. Jonkman, Phd Hendri, H. Pas
    Abstract:

    Background: Pemphigus foliaceus is an Autoimmune Skin Disease characterized by subcorneal blistering and IgG antibodies directed against desmoglein 1. In the Skin, these antibodies deposit intraepidermally. On rare occasions, an additional “lupus band ” of granular depositions of IgG and complement is seen along the epidermal basement membrane zone. This combined pattern has been connected with a variant of pemphigus foliaceus named pemphigus erythematosus. Observations: We describe 3 pemphigus foliaceus cases in which phototherapy was administered after a misdiagnosis of psoriasis. This treatment resulted in a flare of Skin lesions. Direct immunofluorescence of Skin biopsy specimens that were obtained several weeks later demonstrated intraepidermal and granular basement membrane zone depositions. The basement membrane zone depositions consisted of IgG, complement, and the ectodomain of desmoglein 1 and were located below the lamina densa. Conclusions: High doses of UV light are likely to induce the cleaving of the desmoglein 1 ectodomain. In patients with pemphigus foliaceus, the circulating anti– desmoglein 1 antibodies precipitate this cleaved-off ectodomain along the basement membrane zone, resulting in a lupus band–like appearance. In pemphigus erythematosus, a similar mechanism may be active, which might explain the lupus-band phenomenon

Kaisa Tasanen - One of the best experts on this subject based on the ideXlab platform.

  • pemphigus foliaceus and pemphigus erythematosus are the most common subtypes of pemphigus in northern finland
    Acta Dermato-venereologica, 2019
    Co-Authors: Annakaisa Forsti, Ossi Vuorre, Eveliina Laurila, Jari Jokelainen, Laura Huilaja, Kaisa Tasanen
    Abstract:

    Pemphigus is an Autoimmune Skin Disease characterized by blistering and erosions of the Skin and mucous membranes. Pemphigus is rare in Northern and Western Europe but its incidence is higher around the Mediterranean Sea. The most common type worldwide is pemphigus vulgaris. The aim of this study was to investigate the incidence of pemphigus subtypes in Northern Finland between 1985 and 2017. A total of 46 patients diagnosed with pemphigus at the Department of Dermatology of Oulu University Hospital were found the female/male ratio was 1.7. In contrast to many other countries it was found that in Northern Finland the superficial pemphigus subtypes were the most common: erythematosus or foliaceus (65%) followed by pemphigus vulgaris (26%). Over the past 4 decades the annual incidence of pemphigus in Finland has increased from 0.76 to 2.8 cases per million persons.

  • cyclosporine treatment in severe gestational pemphigoid
    Acta Dermato-venereologica, 2015
    Co-Authors: Laura Huilaja, Kaarin Makikallio, Katariina Hannulajouppi, Liisa Vakeva, Johanna Hooknikanne, Kaisa Tasanen
    Abstract:

    Gestational pemphigoid, a rare Autoimmune Skin Disease typically occurring during pregnancy, is caused by autoantibodies against collagen XVII. Clinically it is characterised by severe itching followed by erythematous and bullous lesions of the Skin. Topical or oral glucocorticoids usually relieve symptoms, but in more severe cases systemic immunosuppressive treatments are needed. Data on immunosuppressive medication controlling gestational pemphigoid are sparse. We report 3 intractable cases of gestational pemphigoid treated with cyclosporine.

Gilles F. H. Diercks - One of the best experts on this subject based on the ideXlab platform.

  • The IgG "Lupus-Band" Deposition Pattern of Pemphigus Erythematosus Association With the Desmoglein 1 Ectodomain as Revealed by 3 Cases
    Archives of dermatology, 2012
    Co-Authors: Dyah A. M. Oktarina, Gilles F. H. Diercks, Marcel F. Jonkman, Angelique Poot, Duco Kramer, Hendri H. Pas
    Abstract:

    Background Pemphigus foliaceus is an Autoimmune Skin Disease characterized by subcorneal blistering and IgG antibodies directed against desmoglein 1. In the Skin, these antibodies deposit intraepidermally. On rare occasions, an additional “lupus band” of granular depositions of IgG and complement is seen along the epidermal basement membrane zone. This combined pattern has been connected with a variant of pemphigus foliaceus named pemphigus erythematosus. Observations We describe 3 pemphigus foliaceus cases in which phototherapy was administered after a misdiagnosis of psoriasis. This treatment resulted in a flare of Skin lesions. Direct immunofluorescence of Skin biopsy specimens that were obtained several weeks later demonstrated intraepidermal and granular basement membrane zone depositions. The basement membrane zone depositions consisted of IgG, complement, and the ectodomain of desmoglein 1 and were located below the lamina densa. Conclusions High doses of UV light are likely to induce the cleaving of the desmoglein 1 ectodomain. In patients with pemphigus foliaceus, the circulating anti–desmoglein 1 antibodies precipitate this cleaved-off ectodomain along the basement membrane zone, resulting in a lupus band–like appearance. In pemphigus erythematosus, a similar mechanism may be active, which might explain the lupus-band phenomenon.

  • ONLINE FIRST OBSERVATION The IgG “Lupus-Band ” Deposition Pattern
    2012
    Co-Authors: Of Pemphigus Erythematosus, Gilles F. H. Diercks, Dyah A. M. Oktarina, Phd Angelique, M. Poot, Md Duco Kramer, Arch Dermatol
    Abstract:

    Background: Pemphigus foliaceus is an Autoimmune Skin Disease characterized by subcorneal blistering and IgG antibodies directed against desmoglein 1. In the Skin, these antibodies deposit intraepidermally. On rare occasions, an additional “lupus band ” of granular depositions of IgG and complement is seen along the epidermal basement membrane zone. This combined pattern has been connected with a variant of pemphigus foliaceus named pemphigus erythematosus. Observations: We describe 3 pemphigus foliaceus cases in which phototherapy was administered after a misdiagnosis of psoriasis. This treatment resulted in a flare of Skin lesions. Direct immunofluorescence of Skin biopsy specimens that were obtained several weeks later demonstrated intraepidermal and granular basement membrane zone depositions. The basement membrane zone depositions consisted of IgG, complement, and the ectodomain of desmoglein 1 and were located below the lamina densa. Conclusions: High doses of UV light are likely to induce the cleaving of the desmoglein 1 ectodomain. In patients with pemphigus foliaceus, the circulating anti– desmoglein 1 antibodies precipitate this cleaved-off ectodomain along the basement membrane zone, resulting in a lupus band–like appearance. In pemphigus erythematosus, a similar mechanism may be active, which might explain the lupus-band phenomenon

  • Association With the Desmoglein 1 Ectodomain as Revealed by 3 Cases
    2012
    Co-Authors: Of Pemphigus Erythematosus, Gilles F. H. Diercks, Dyah A. M. Oktarina, Phd Angelique, M. Poot, Md Duco Kramer, Phd Marcel, F. Jonkman, Phd Hendri, H. Pas
    Abstract:

    Background: Pemphigus foliaceus is an Autoimmune Skin Disease characterized by subcorneal blistering and IgG antibodies directed against desmoglein 1. In the Skin, these antibodies deposit intraepidermally. On rare occasions, an additional “lupus band ” of granular depositions of IgG and complement is seen along the epidermal basement membrane zone. This combined pattern has been connected with a variant of pemphigus foliaceus named pemphigus erythematosus. Observations: We describe 3 pemphigus foliaceus cases in which phototherapy was administered after a misdiagnosis of psoriasis. This treatment resulted in a flare of Skin lesions. Direct immunofluorescence of Skin biopsy specimens that were obtained several weeks later demonstrated intraepidermal and granular basement membrane zone depositions. The basement membrane zone depositions consisted of IgG, complement, and the ectodomain of desmoglein 1 and were located below the lamina densa. Conclusions: High doses of UV light are likely to induce the cleaving of the desmoglein 1 ectodomain. In patients with pemphigus foliaceus, the circulating anti– desmoglein 1 antibodies precipitate this cleaved-off ectodomain along the basement membrane zone, resulting in a lupus band–like appearance. In pemphigus erythematosus, a similar mechanism may be active, which might explain the lupus-band phenomenon

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