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1/12. Dysphagia in a patient with lupus and review of the literature.

    Dysphagia is not infrequent in patients with connective tissue diseases such as scleroderma, polymyositis or systemic lupus erythematosus (SLE). It is usually the result of gastro-oesophageal reflux but dysmotility can equally be responsible. A case of dysphagia is described in a patient with SLE, who had developed a rare variety of bullous mucous disease affecting the whole length of oesophagus with spontaneous extrusion of an oesophageal cast. Histological features were suggestive of a variant of rare form of bullous disease in SLE called epidermolysis bullosa acquisita (EBA). This rare association of SLE and EBA involving the oesophagus has not been described in the literature.
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ranking = 1
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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2/12. Bullous systemic lupus erythematosus with milia and calcinosis.

    Bullous systemic lupus erythematosus (SLE) is a rare skin manifestation of SLE. It shares many features with epidermolysis bullosa acquisita (EBA). We report on a patient with SLE who developed a vesiculobullous eruption followed by findings not typical in bullous SLE, namely milia, mild scarring, and calcinosis. We discuss the relationship between bullous SLE and EBA.
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ranking = 1
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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3/12. Detection of anti-basement membrane zone antibodies in bullous systemic lupus erythematosus.

    We describe a 42-year-old black woman with long-standing systemic lupus erythematosus in whom vesiculobullous lesions developed. Routine histologic and immunologic studies fulfilled the criteria for the diagnosis of bullous systemic lupus erythematosus. Indirect immunofluorescence showed antinuclear antibodies without basement membrane zone fluorescence. We destroyed the nuclear antigens of the indirect immunofluorescence substrate with 2 mol/L sodium chloride, which unmasked basement membrane zone linear IgG staining. We also confirmed anti-basement membrane zone antibodies by employing a new technique of direct immunofluorescence on sodium chloride-split skin. Our finding prove that a thorough search for anti-basement membrane zone antibodies can be revealing. Our results support the idea that a subset of bullous systemic lupus erythematosus has the staining characteristics of epidermolysis acquisita, with the dermal side of the split skin showing linear immunoglobulin deposition.
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ranking = 0.056287498410501
keywords = acquisita, epidermolysis
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4/12. epidermolysis bullosa acquisita preceding the development of systemic lupus erythematosus.

    In most cases of epidermolysis bullosa acquisita that occur in patients with systemic lupus erythematosus, the diagnosis of systemic lupus erythematosus is made before the development of blistering. We observed three patients with well-documented epidermolysis bullosa acquisita that developed several years before the onset of systemic lupus erythematosus. One patient was producing anti-U1RNP autoantibodies at the time epidermolysis bullosa acquisita was diagnosed, and all five produced this antibody during the systemic lupus erythematosus phase of their illness. In addition, in all five cases of epidermolysis bullosa acquisita with systemic lupus erythematosus antibodies to double-stranded dna ultimately developed, and severe systemic lupus erythematosus and lupus nephritis developed in four patients. Sera from 15 other patients with epidermolysis bullosa acquisita without overt systemic lupus erythematosus were analyzed for systemic lupus erythematosus-related autoantibodies. Four patients were found to have at least one such autoantibody. These findings further document an association between epidermolysis bullosa acquisita and systemic lupus erythematosus and suggest that patients with systemic lupus erythematosus who present with epidermolysis bullosa acquisita may represent a subset of lupus erythematosus that puts the patient at increased risk for the development of more severe systemic illness. patients presenting with epidermolysis bullosa acquisita, especially those who are black or Hispanic, should be monitored for the development of potentially life-threatening systemic lupus erythematosus.
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ranking = 8.8807538022977
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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5/12. Systemic lupus erythematosus presenting as a bullous eruption in a child.

    An 8-year-old girl presented with a generalized bullous eruption clinically resembling bullous pemphigoid or chronic bullous disease of childhood. Further study revealed immunopathologic findings seen in patients with epidermolysis bullosa acquisita or bullous systemic lupus erythematosus (SLE). Although she did not fulfill the American Rheumatism association (Atlanta) criteria for SLE at her presentation, one year later she went on to do so. As well as being the youngest patient reported with bullous SLE, our patient is noteworthy because the bullous eruption was the initial manifestation of her SLE. Bullous SLE should be considered in the differential diagnosis of children presenting with generalized bullous eruptions.
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ranking = 1
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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6/12. Bullous systemic lupus erythematosus: an unusual clinical course and detectable circulating autoantibodies to the epidermolysis bullosa acquisita antigen.

    Bullous systemic lupus erythematosus is a newly recognized form of systemic lupus erythematosus characterized by a skin eruption clinically and histologically resembling dermatitis herpetiformis and responsive to dapsone. We report on a patient with bullous systemic lupus erythematosus who initially presented with lesions clinically resembling erythema multiforme, experienced exacerbation of her disease with dapsone, and had detectable circulating autoantibodies to the epidermolysis bullosa acquisita antigen.
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ranking = 5
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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7/12. Evidence that anti-basement membrane zone antibodies in bullous eruption of systemic lupus erythematosus recognize epidermolysis bullosa acquisita autoantigen.

    Circulating and tissue-deposited IgG antibodies to the cutaneous basement membrane zone (BMZ) were detected in 3 patients with the clinical, pathologic, and immunologic features of bullous eruption of systemic lupus erythematosus (SLE). The antibodies were present in sera and IgG fractions in all cases and in eluates of cutaneous immune deposits from one of the cases. The antibodies were easily detected in sera by indirect immunofluorescence on adult human thigh skin separated through the lamina lucida by incubation in 1.0 M NaCl but were less easily detected on intact neonatal foreskin. The antibodies had features of epidermolysis bullosa acquisita (EBA) anti-BMZ antibodies including binding to the dermal side of the BMZ in separated skin, binding to the cutaneous but not vascular or glomerular basement membranes, binding to and just below the lamina densa, and binding to 290 or 290 and 145 kD dermal proteins previously identified as components of the EBA autoantigen. The antibodies were relatively specific for SLE patients with features of bullous eruption of SLE since they were detected in 3 of 4 of those cases and in only 1 of 20 SLE patients without blisters. These results show anti-BMZ antibodies with features of EBA antibodies are present in patients with bullous eruption of SLE and suggest there may be a close relationship between that disease and EBA. The results also suggest that EBA antibodies may be part of the autoantibody spectrum of SLE and that separated skin is more sensitive than intact skin for their detection.
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ranking = 5
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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8/12. Systemic lupus erythematosus occurring in a patient with epidermolysis bullosa acquisita.

    A dystrophic bullous eruption that met all the criteria for epidermolysis bullosa acquisita (EBA) developed in a 19-year-old woman five years before the onset of clinical and serologic evidence of systemic lupus erythematosus (SLE). Electron microscopic studies of skin lesions both before and after the development of SLE were consistent with the previously reported electron microscopic findings in patients with EBA. Direct immunofluorescence microscopic studies done on bullae before and after the diagnosis of SLE showed linear depositions of immunoglobulin and complement; indirect immunofluorescence microscopic study findings consistently showed no abnormalities. These findings have been noted in other cases of EBA and may implicate autoimmune, immunologic factors in the pathogenesis of the disease process. To our knowledge, the finding of SLE in association with EBA has not been previously reported.
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ranking = 5
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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9/12. Fatal vascular involvement in systemic lupus erythematosus following epidermolysis bullosa acquisita.

    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.
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ranking = 7.2201884505744
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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10/12. epidermolysis bullosa acquisita occurring in association with systemic lupus erythematosus.

    A 77-year-old retired male physician with a 6-year history of systemic lupus erythematosus (SLE) developed a mechanobullous eruption, the features of which were clinically and immunopathologically consistent with a diagnosis of 'classical' epidermolysis bullosa acquisita (EBA). As EBA shares immunopathological findings with a number of cases reported as the 'bullous eruption of SLE', the clinical findings commonly recognized as 'classical EBA' may, in patients with SLE, represent a specific subset of the bullous eruption of SLE rather than a separate diagnostic entity. There are few reports in the literature describing classical EBA in patients with SLE. Findings in this patient add further support to the suggestion that EBA occurring in association with SLE, represents a subset of the bullous eruption of SLE, the clinical features of which may be modified by genetic susceptibility or disease activity.
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ranking = 1.8807538022977
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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