Cases reported "Autoimmune Diseases"

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1/22. Subepidermal autoimmune bullous skin diseases associated with B-cell lymphoproliferative disorders.

    The development of several types of autoimmune disorders is well known in the course of B-cell lymphoproliferative diseases. In order to investigate whether these autoimmune diseases may target cutaneous antigens, especially those of the dermal-epidermal junction, we conducted a retrospective analysis of all cutaneous direct immunofluorescence studies performed in patients with B-cell neoplasia who presented with cutaneous lesions requiring a skin biopsy. patients with linear deposits of immunoglobulin or C3 molecules at the dermal-epidermal junction were studied further, using immunoblotting and immunoelectron microscopy. patients with no overt hematologic malignancies but with cutaneous lesions requiring direct immunofluorescence were studied as a control population. During the study period, the diagnosis of autoimmune blistering diseases of the dermal-epidermal junction was established in 9/102 (8.9% /- 2%) patients with a B-cell lymphoid disorder, compared with 288/8,189 (3.5% /- 0.04%) patients without underlying hemopathy (p < 0.01). Among the 9 patients, 7 were studied in detail. Immunologic studies demonstrated epidermolysis bullosa acquisita in 6 patients and cicatricial pemphigoid in 1. Autoimmune blistering diseases, especially epidermolysis bullosa acquisita, are part of the spectrum of autoimmune disorders associated with B-cell neoplasia.
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ranking = 1
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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2/22. A case of acquired autoimmune bullous disease associated with IgM macroglobulinaemia.

    A variety of autoimmune bullous dermatoses have been reported to develop in association with lymphoproliferative disorders. We report a patient with IgM macroglobulinaemia, who presented with a skin fragility similar to but somewhat milder than that seen in epidermolysis bullosa acquisita. Immunofluorescence detected circulating IgM autoantibodies reacting with the basement membrane zone, which reacted predominantly with dermal side of 1 M NaCl-split skin. immunoblotting of the epidermal and dermal extracts with the patient's serum showed no specific reactivity. Further studies are needed to identify the antigenic molecules responsible for the IgM deposition.
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ranking = 0.5
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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3/22. Ocular involvement in IgA-epidermolysis bullosa acquisita.

    epidermolysis bullosa acquisita (EBA) is an autoimmune bullous disease with frequent ocular involvement, but visual loss is rare. In contrast, EBA patients with predominant IgA autoantibodies more frequently develop severe ocular involvement, which tends to be refractory to therapy. We report two patients with 'IgA-EBA' with ocular involvement. Both initially presented with a generalized bullous disease, and direct immunofluorescence microscopy demonstrated IgA in the basement membrane zone of the skin, and in the conjunctiva and cornea of patient 1. On salt-split patient skin, IgA was found predominantly on the dermal side of the artificial split in both patients. Direct immunoelectron microscopy demonstrated IgA below the lamina densa in close association with the anchoring fibrils in both patients. In patient 1, who had a prolonged course of the disease, the skin disorder responded well to treatment with cyclosporin, but the ocular involvement ended in bilateral blindness despite repeated surgical treatment. In patient 2, the blister formation and scarring conjunctivitis was stopped by a combination of prednisolone and colchicine. These patients show that in subepithelial blistering diseases, early delineation of disease nosology is critical to detect subtypes with severe ocular involvement such as 'IgA-EBA'. In addition, colchicine may be a valuable alternative in the treatment of EBA with ocular involvement.
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ranking = 2.126008394199
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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4/22. A subepidermal bullous eruption associated with IgG autoantibodies to a 200 kd dermal antigen: the first case report from the united states.

    We describe an 81-year-old white man in whom a subepidermal bullous eruption developed that clinically resembled bullous pemphigoid. The eruption promptly responded to oral tetracycline and niacinamide and topical clobetasol. Histologic examination of perilesional skin revealed neutrophilic infiltration with formation of papillary microabscesses and subepidermal cleavage. Direct immunofluorescence showed linear deposition of IgG and C3 along the basement membrane zone. By indirect immunofluorescence, circulating IgG autoantibodies bound exclusively to the dermal side of salt-split normal human skin. Immunoblot analysis demonstrated that the patient's autoantibodies reacted with a 200 kd dermal protein that was different from type VII collagen, the epidermolysis bullosa acquisita autoantigen. This patient represents the first confirmed case from the united states with a recently reported novel autoimmune subepidermal bullous disease associated with IgG autoantibodies to a 200 kd dermal antigen.
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ranking = 0.5
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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5/22. Childhood epidermolysis bullosa acquisita: a novel variant with reactivity to all three structural domains of type VII collagen.

    Most patients with epidermolysis bullosa acquisita develop an autoimmune response to the non-collagenous (NC) 1 domain of type VII collagen. We report a 4-year-old girl of white European descent presenting with widespread blistering disease involving the face, hands, genital area and oral mucosa. Histopathology revealed subepidermal blisters, and linear deposits of IgG and C3 were seen along the dermal-epidermal junction on direct immunofluorescence (IF) microscopy of a perilesional skin biopsy. On indirect IF microscopy, circulating autoantibodies exclusively stained the dermal side of 1 mol L-1 NaCl-split skin. The patient's IgG autoantibodies labelled a 290-kDa protein on Western blotting of dermal extracts, and reacted with the NC1, NC2 and triple helical domains of type VII collagen on immunoblotting of recombinant and cell-derived fragments obtained by pepsin and collagenase digestion of the full-length protein. Oral methylprednisolone and dapsone led to clearance of lesions, which healed with mild scarring and milia formation. Treatment was discontinued after 1 year and the patient has now been in remission for more than 3 years.
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ranking = 2.5
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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6/22. psoriasis bullosa acquisita.

    We report a 51-year-old man with a 20-year history of chronic plaque psoriasis who developed an autoimmune subepidermal blistering eruption that had clinical features of bullous pemphigoid, erythema multiforme and epidermolysis bullosa acquisita. Investigations revealed a 1 : 400 titre circulating and in vivo bound IgG autoantibody that mapped to the dermal side of 1 m NaCl-split skin and localized to the lower lamina lucida/upper lamina densa on immunogold electron microscopy. immunoblotting, using dermal extracts, showed serum binding to antigens of approximately 200- and approximately 260 kDa. Indirect immunofluorescence microscopy, using the patient's serum on archival skin sections taken from selected individuals with different forms of inherited epidermolysis bullosa as substrate, showed normal basement membrane labelling on all samples apart from recessive dystrophic epidermolysis bullosa skin (with inherent mutations in the type VII collagen gene): in these cases there was a complete absence of immunostaining. Clinically, the patient responded rapidly to combination treatment with intravenous immunoglobulin and oral corticosteroids, dapsone and mycophenolate mofetil. Autoimmune subepidermal blistering has been reported in other patients with psoriasis, although no specific target antigen has ever been determined. Our study provides preliminary evidence that, for this patient at least, the autoantibody may be targeted against a skin component closely associated with type VII collagen (the epidermolysis bullosa acquisita antigen). Therefore, we propose the term 'psoriasis bullosa acquisita' for this and possibly other patients with similar skin eruptions.
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ranking = 1.7084198405642
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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7/22. Anti-interferon-gamma antibodies in the treatment of autoimmune diseases.

    Interferon (IFN)-gamma is an important immune regulator in normal immunity. When IFN gamma production is disturbed, various autoimmune diseases (ADs) can develop, in which we suggest that anti-IFN gamma could have a beneficial effect. Depending on the cell type in which IFN gamma synthesis is disturbed, different clinical manifestations may result. We have also proposed to remove tumor necrosis factor (TNF)-alpha, together with certain types of IFNs, to treat various ADs and AIDS, also an autoimmune condition. Anti-IFN gamma has been tested in several T-helper cell (Th1) ADs, including rheumatoid arthritis (RA), multiple sclerosis (MS), corneal transplant rejection, uveitis, Type I diabetes, schizophrenia (anti-IFN gamma and anti-TNF alpha), and various autoimmune skin diseases (alopecia areata, psoriasis vulgaris, vitiligo, pemphigus vulgaris and epidermolysis bullosa). A strong, sometimes striking, therapeutic response followed administration of anti-IFN gamma, indicating that it may be a promising therapy for Th1 ADs.
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ranking = 0.039188934784691
keywords = epidermolysis bullosa, epidermolysis, bullosa
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8/22. epidermolysis bullosa acquisita, a rare late complication of allogeneic bone marrow transplantation?

    We report a rare disease of skin and oropharyngeal mucosa in a 28-year-old patient occurring 2 years after an allogeneic bone marrow transplantation. The dermatologic diagnosis was unambiguously epidermolysis bullosa acquisita according to the immunofluorescence and clinical presentation. Treatment with cyclosporin A and prednisone resulted in resolution. This autoimmune skin disease may be a manifestation of graft-versus-host disease, but the relationship must remain speculative.
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ranking = 1.0040335767958
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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9/22. A case of mixed bullous disease of epidermolysis bullosa acquisita and linear iga bullous dermatosis.

    A 75-year-old Japanese male visited us with bullous eruptions on the extremities. physical examination revealed large bullae on the hands, lower legs and feet. The oral mucosa was also involved. histology disclosed subepidermal blister with inflammatory cell infiltrates in the dermis. Direct immunofluorescence showed deposits of IgG and IgA at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient's IgG antibodies reacted with the dermal side of the split, while IgA antibodies reacted with the epidermal side. immunoblotting showed that the patient's serum reacted with the NC1 domain of type VII collagen (290-kDa epidermolysis bullosa acquisita antigen) as well as the 120-kDa linear iga bullous dermatosis antigen, LAD-1. Systemic prednisolone resulted in a favorable response. From the clinicopathological findings, the present case is not consistent with either epidermolysis bullosa acquisita or IgA bullous dermatosis. Therefore, we regarded the case as mixed bullous disease of epidermolysis bullosa acquisita and linear iga bullous dermatosis. Such a case has not been previously reported.
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ranking = 3.5
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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10/22. epidermolysis bullosa acquisita in a patient with multiple endocrinopathies syndrome.

    epidermolysis bullosa acquisita is an acquired blistering disorder, with a probable autoimmune basis, which has been associated with a number of other autoimmune disorders. We encountered a case of epidermolysis bullosa acquisita associated with multiple endocrinopathies syndrome.
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ranking = 1.1300419709948
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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