The Experts below are selected from a list of 225 Experts worldwide ranked by ideXlab platform
David T Woodley - One of the best experts on this subject based on the ideXlab platform.
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Epidermolysis Bullosa Acquisita
Clinics in Dermatology, 2012Co-Authors: Rishu Gupta, David T Woodley, Mei ChenAbstract:Abstract Epidermolysis Bullosa Acquisita (EBA) is a rare, acquired, chronic subepidermal bullous disease of the skin and mucosa characterized by autoantibodies to type VII collagen (C7) structures, a major component of anchoring fibrils, which attach the epidermis to the dermis. EBA patients have tissue-bound and circulating antitype C7 autoantibodies that attack type C7 and result in a reduction or perturbation of normally functioning anchoring fibrils. Patients with EBA have skin fragility, blisters, erosions, scars, milia, and nail loss, all features reminiscent of genetic dystrophic Epidermolysis Bullosa. These immunoglobulin G antitype C7 antibodies are pathogenic, because when they are injected into mice, the mice develop an EBA-like blistering disease. In addition to the classical mechanobullous presentation, EBA also has several other distinct clinical syndromes similar to bullous pemphigoid, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. Although treatment for EBA is often unsatisfactory, some therapeutic success has been achieved with colchicine, dapsone, plasmapheresis, photopheresis, infliximab, and intravenous immunoglobulin.
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Familial Epidermolysis Bullosa Acquisita.
Dermatology Online Journal, 2008Co-Authors: Mei Chen, David T Woodley, Janet A. FairleyAbstract:Epidermolysis Bullosa Acquisita (EBA) is an acquired blistering skin disorder caused by IgG autoantibodies directed against type VII collagen. In contrast to the genetic forms of Epidermolysis Bullosa, EBA is usually an acquired, sporadic disease. In this report, we describe a family with two cases of EBA in an uncle-nephew pair, and a third family member with asymptomatic circulating anti-type VII collagen antibodies. These findings provide support for the hypothesis that there is a genetic component to EBA.
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Autoimmunity to type VII collagen: Epidermolysis Bullosa Acquisita.
Clinical Reviews in Allergy & Immunology, 2007Co-Authors: David T Woodley, Jennifer Remington, Mei ChenAbstract:Epidermolysis Bullosa Acquisita (EBA) is an acquired, mechanobullous disease characterized by autoimmunity to type VII collagen. Type VII collagen makes anchoring fibrils, structures that connect th
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A patient with both bullous pemphigoid and Epidermolysis Bullosa Acquisita: an example of intermolecular epitope spreading.
Journal of the American Academy of Dermatology, 2004Co-Authors: Janet A. Fairley, David T Woodley, Mei Chen, George J. Giudice, Mong-shang LinAbstract:Bullous pemphigoid (BP) and Epidermolysis Bullosa Acquisita are distinct autoimmune blistering disorders. BP is characterized by autoantibodies directed against the NC16A domain of collagen XVII, whereas patients with Epidermolysis Bullosa Acquisita have autoantibodies against the NC1 domain of type VII collagen. We followed up a patient with BP for 9 years. During that time his clinical disease took on several features suggestive of Epidermolysis Bullosa Acquisita. The objective of this study was to determine if the patient's autoantibody profile reflected the change in his clinical picture. Enzyme-linked immunosorbent assay and immunoblotting for detection and subclass determination of autoantibodies to type XVII and type VII collagen were performed on banked patient sera from the 9-year period. The patient's initial autoantibodies were exclusively IgG1 directed against collagen XVII. During the course of his illness, the subclass specificity of the patient's type XVII collagen autoantibodies shifted to the IgG4 subclass and during the same time interval the patient developed IgG2 autoantibodies directed against type VII collagen. This patient with BP exhibited both subclass shifting and development of a second autoantibody system that correlated with a change in the clinical appearance of the disease. The analysis of the patient's autoantibodies provides strong evidence for the involvement of epitope spreading in the evolution of his autoimmune disease.
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Epidermolysis Bullosa Acquisita in Childhood
The Journal of dermatology, 2003Co-Authors: Fanny X. Trigo-guzmán, David T Woodley, Adriana Conti, Valeria Aoki, Celina Wakisaka Maruta, Claudia Giuli Santi, Cláudia Márcia De Resende Silva, Bernardo Gontijo, Evandro A. RivittiAbstract:Epidermolysis Bullosa Acquisita (EBA) is a subepidermal autoimmune blistering disease that is rarely reported in childhood. We describe a nine-month-old mulatto boy presenting with multiple, annular, widespread, tense blisters and oral lesions. The diagnosis of EBA was confirmed by histopathology, immunofluorescence, and immunoblotting analysis. The patient was successfully treated with systemic steroids (prednisone) and dapsone. After 20 months of initial treatment, clinical remission was observed, and dapsone remains as the current treatment. This case report emphasizes the rarity of EBA in childhood and the difficulties in reaching the final diagnosis.
Soo-chan Kim - One of the best experts on this subject based on the ideXlab platform.
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Epidermolysis Bullosa Acquisita
Journal of the European Academy of Dermatology and Venereology : JEADV, 2013Co-Authors: Jong Hoon Kim, Soo-chan KimAbstract:Epidermolysis Bullosa Acquisita (EBA) is a chronic autoimmune subepidermal bullous disease with clinical features similar to the genetic form of dystrophic Epidermolysis Bullosa. EBA is characterized by the presence of autoantibodies against type VII collagen which is a major component of the anchoring fibrils at the dermal-epidermal junction. EBA can be divided into two main clinical types; mechanobullous and inflammatory EBA. Mechanobullous EBA, referred to as classic EBA, presents with skin fragility, blisters and dystrophic changes on trauma-prone areas. Inflammatory EBA resembles other autoimmune subepidermal bullous diseases. Compelling evidence from mouse models supports a pathogenic role of autoantibodies against type VII collagen in EBA. Treatment of EBA is often unsatisfactory. The most widely used systemic treatment is corticosteroids. Colchicine and dapsone have been reported to be good treatment modalities when combined with corticosteroids. Some intractable cases of EBA have successfully been treated with intravenous immunoglobulin or rituximab.
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Epidermolysis Bullosa Acquisita a retrospective clinical analysis of 30 cases
Acta Dermato-venereologica, 2011Co-Authors: Jong Hoon Kim, Yeon Hee Kim, Soo-chan KimAbstract:Epidermolysis Bullosa Acquisita (EBA) is an acquired, autoimmune blistering disorder caused by autoantibody production against type VII collagen. The aim of this study was to examine the clinical types, treatments, and outcomes of 30 patients with EBA. In our cohort, the median age of onset was 44.0 years, with a similar incidence for both genders (46.7% male, 53.3% female). The majority of patients had classic type (36.7%) and bullous pemphigoid (BP)-like type (46.7%) EBA. The remaining patients had mucous membrane pemphigoid-like (6.7%), Brunsting-Perry pemphigoid-like (6.7%), and linear IgA bullous dermatosis-like type (3.3%) EBA. All patients were treated initially with a combination of methylprednisolone, dapsone and colchicine. No significant differences in time to remission were identified between patients with classic vs. BP-like EBA. In a second subset analysis of 19 patients, a group treated with high-dose (> 8 mg) methylprednisolone achieved remission earlier (median time to remission: 3 months) than a group treated with low-dose (≤ 8 mg) methylprednisolone (median time to remission: 12 months), irrespective of clinical type (p = 0.003). Key words: Epidermolysis Bullosa Acquisita; retrospective study, clinical study remission.
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Fatal vascular involvement in systemic lupus erythematosus following Epidermolysis Bullosa Acquisita.
Acta dermato-venereologica, 1995Co-Authors: Juho Yoon, Tae Kee Moon, Kwang Hoon Lee, Soo-chan KimAbstract:Epidermolysis Bullosa Acquisita may be associated with various systemic diseases, including systemic lupus erythematosus. We describe the clinical and immunological findings in a 38-year-old women with Epidermolysis Bullosa Acquisita and systemic lupus erythematosus. The Epidermolysis Bullosa Acquisita preceded a dramatic flare of systemic lupus erythematosus and fatal cerebral vasculitis. If serologic evidence of lupus erythematosus develops during the course of Epidermolysis Bullosa Acquisita, a thorough investigation is warranted to rule out potentially life-threatening systemic lupus erythematosus.
Mei Chen - One of the best experts on this subject based on the ideXlab platform.
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Epidermolysis Bullosa Acquisita
Clinics in Dermatology, 2012Co-Authors: Rishu Gupta, David T Woodley, Mei ChenAbstract:Abstract Epidermolysis Bullosa Acquisita (EBA) is a rare, acquired, chronic subepidermal bullous disease of the skin and mucosa characterized by autoantibodies to type VII collagen (C7) structures, a major component of anchoring fibrils, which attach the epidermis to the dermis. EBA patients have tissue-bound and circulating antitype C7 autoantibodies that attack type C7 and result in a reduction or perturbation of normally functioning anchoring fibrils. Patients with EBA have skin fragility, blisters, erosions, scars, milia, and nail loss, all features reminiscent of genetic dystrophic Epidermolysis Bullosa. These immunoglobulin G antitype C7 antibodies are pathogenic, because when they are injected into mice, the mice develop an EBA-like blistering disease. In addition to the classical mechanobullous presentation, EBA also has several other distinct clinical syndromes similar to bullous pemphigoid, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. Although treatment for EBA is often unsatisfactory, some therapeutic success has been achieved with colchicine, dapsone, plasmapheresis, photopheresis, infliximab, and intravenous immunoglobulin.
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Familial Epidermolysis Bullosa Acquisita.
Dermatology Online Journal, 2008Co-Authors: Mei Chen, David T Woodley, Janet A. FairleyAbstract:Epidermolysis Bullosa Acquisita (EBA) is an acquired blistering skin disorder caused by IgG autoantibodies directed against type VII collagen. In contrast to the genetic forms of Epidermolysis Bullosa, EBA is usually an acquired, sporadic disease. In this report, we describe a family with two cases of EBA in an uncle-nephew pair, and a third family member with asymptomatic circulating anti-type VII collagen antibodies. These findings provide support for the hypothesis that there is a genetic component to EBA.
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Autoimmunity to type VII collagen: Epidermolysis Bullosa Acquisita.
Clinical Reviews in Allergy & Immunology, 2007Co-Authors: David T Woodley, Jennifer Remington, Mei ChenAbstract:Epidermolysis Bullosa Acquisita (EBA) is an acquired, mechanobullous disease characterized by autoimmunity to type VII collagen. Type VII collagen makes anchoring fibrils, structures that connect th
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A patient with both bullous pemphigoid and Epidermolysis Bullosa Acquisita: an example of intermolecular epitope spreading.
Journal of the American Academy of Dermatology, 2004Co-Authors: Janet A. Fairley, David T Woodley, Mei Chen, George J. Giudice, Mong-shang LinAbstract:Bullous pemphigoid (BP) and Epidermolysis Bullosa Acquisita are distinct autoimmune blistering disorders. BP is characterized by autoantibodies directed against the NC16A domain of collagen XVII, whereas patients with Epidermolysis Bullosa Acquisita have autoantibodies against the NC1 domain of type VII collagen. We followed up a patient with BP for 9 years. During that time his clinical disease took on several features suggestive of Epidermolysis Bullosa Acquisita. The objective of this study was to determine if the patient's autoantibody profile reflected the change in his clinical picture. Enzyme-linked immunosorbent assay and immunoblotting for detection and subclass determination of autoantibodies to type XVII and type VII collagen were performed on banked patient sera from the 9-year period. The patient's initial autoantibodies were exclusively IgG1 directed against collagen XVII. During the course of his illness, the subclass specificity of the patient's type XVII collagen autoantibodies shifted to the IgG4 subclass and during the same time interval the patient developed IgG2 autoantibodies directed against type VII collagen. This patient with BP exhibited both subclass shifting and development of a second autoantibody system that correlated with a change in the clinical appearance of the disease. The analysis of the patient's autoantibodies provides strong evidence for the involvement of epitope spreading in the evolution of his autoimmune disease.
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Epidermolysis Bullosa Acquisita: update and review.
Clinics in dermatology, 2001Co-Authors: Dafna Hallel-halevy, Mei Chen, Celina Nadelman, David T WoodleyAbstract:Epidermolysis Bullosa Acquisita (EBA) is a chronic subepidermal bullous disease of the skin and mucosa characterized by the presence of autoantibodies to type VII collagen, a major component of anchoring fibrils, structures that help adhere the epidermis onto the dermis. EBA was classified as an Epidermolysis Bullosa (EB) disease because clinically it can resemble hereditary dystrophic forms of EB; yet, it has a different etiology and is an acquired disease rather than a genetic one.
C Prost - One of the best experts on this subject based on the ideXlab platform.
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Epidermolysis Bullosa Acquisita diagnosed by direct immunoelectron microscopy of the conjunctiva
Ophthalmology, 1997Co-Authors: Thanh Hoang-xuan, Hervé Robin, Michel Heller, Frédéric Caux, C ProstAbstract:Objective: To describe for the first time the direct immunoelectron microscopic pattern of immune deposits on the conjunctival basement membrane in Epidermolysis Bullosa Acquisita (EBA). Design: Case reports. Participants: Two patients. Intervention: Epidermolysis Bullosa Acquisita associated with cicatrizing conjunctivitis. Main Outcome Measures: Direct immunofluorescence and direct immunoelectron microscopy without freezing on conjunctival and skin biopsy specimens, indirect immunofluorescence, Western immunoblot analysis. Results: Results of direct immunoelectron microscopic examination of the conjunctiva showed the presence of immune deposits in the anchoring fibril zone, just beneath the lamina densa, in both patients. This finding was the same as the direct immunoelectron microscopic pattern shown in the skin of these patients, which is known to be very specific for EBA. Direct immunofluorescence was positive in the conjunctiva of only one patient. Indirect immunofluorescence and Western immunoblot analysis failed to detect circulating autoantibodies. Conclusions: Direct immunoelectron microscopy on the conjunctiva is a useful diagnostic tool to differentiate EBA from other related autoimmune mucocutaneous blistering diseases.
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Epidermolysis Bullosa Acquisita in a 3 1/2-year-old girl.
Journal of the American Academy of Dermatology, 1992Co-Authors: H Roger, Jean Kanitakis, P Machado, J F Nicolas, M D'incan, C Prost, C Chevenet, M C Ferrier, B Chouvet, P SouteyrandAbstract:A 3 1/2-year-old girl had a subepidermal bullous eruption with immunopathologic features that were consistent with Epidermolysis Bullosa Acquisita or bullous systemic lupus erythematosus. This report highlights the difficulty encountered in distinguishing between Epidermolysis Bullosa Acquisita and other bullous disorders that involve the dermoepidermal junction and the need for modern immunologic investigations in the diagnosis of bullous diseases in children.
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Epidermolysis Bullosa Acquisita in a 3½-year-old girl
Journal of the American Academy of Dermatology, 1992Co-Authors: H Roger, Jean Kanitakis, J F Nicolas, M D'incan, C Prost, C Chevenet, M C Ferrier, B Chouvet, Paulo Roberto Lima Machado, P SouteyrandAbstract:A 3½-year-old girl had a subepidermal bullous eruption with immunopathologic features that were consistent with Epidermolysis Bullosa Acquisita or bullous systemic lupus erythematosus. This report highlights the difficulty encountered in distinguishing between Epidermolysis Bullosa Acquisita and other bullous disorders that involve the dermoepidermal junction and the need for modern immunologic investigations in the diagnosis of bullous diseases in children.
Fujio Otsuka - One of the best experts on this subject based on the ideXlab platform.
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Autoantibodies to bullous pemphigoid and Epidermolysis Bullosa Acquisita antigens in an infant
The British journal of dermatology, 1996Co-Authors: Yasuhiro Kawachi, M. Ikegami, Takashi Hashimoto, K. Matsumura, T. Tanaka, Fujio OtsukaAbstract:We describe a 1-year-old boy with multiple tense blisters on the skin, who showed circulating autoantibodies directed to both bullous pemphigoid and Epidermolysis Bullosa Acquisita antigens. The patient's serum IgG antibodies bound to the 290-kDa Epidermolysis Bullosa Acquisita antigen with immunoblot analysis of human dermal extracts. Immunoblot analysis also demonstrated that the patient's serum autoantibodies were reactive with recombinant NC16a domain of the 180-kDa bullous pemphigoid antigen. This study confirmed the presence of circulating autoantibodies directed to both bullous pemphigoid antigen and Epidermolysis Bullosa Acquisita antigen.