Cases reported "Esophageal Diseases"

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1/10. epidermolysis bullosa acquisita (EBA) and esophageal webs: a new association.

    epidermolysis bullosa acquisita (EBA) is a well-defined, blistering disorder of the skin associated with autoantibodies to type VII collagen. Although esophageal pathology is common in children with hereditary dystrophic forms of epidermolysis bullosa, esophageal problems have not been reported previously in patients with bona fide EBA. In this report, a 71-yr-old white female with longstanding EBA presented with recurrent dysphagia and multiple esophageal webs that responded to esophageal dilatation.
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ranking = 1
keywords = epidermolysis bullosa, bullosa acquisita, epidermolysis, acquisita, bullosa
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2/10. epidermolysis bullosa acquisita and associated symptomatic esophageal webs.

    epidermolysis bullosa acquisita (EBA) is a well-characterized, subepidermal blistering disorder associated with autoimmunity to type VII collagen, which is the collagen localized to anchoring fibrils within the dermoepidermal junction of skin. Although the full clinical spectrum of EBA is still being defined, it is known that the clinical features of EBA may be reminiscent of hereditary dystrophic epidermolysis bullosa, a scarring blistering disease of children that is commonly associated with esophageal stenosis. We describe a patient with EBA who had both an acral-predominant mechanobullous disease akin to dystrophic epidermolysis bullosa and an inflammatory, widespread bullous eruption reminiscent of bullous pemphigoid in association with esophageal webs and dysphagia. Although esophageal involvement is common in dystrophic epidermolysis bullosa, a review of the literature shows that this is the first bonafide case of EBA with symptomatic esophageal disease.
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ranking = 1.3888299745304
keywords = epidermolysis bullosa, bullosa acquisita, epidermolysis, acquisita, bullosa
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3/10. Gastrointestinal manifestations of epidermolysis bullosa in children.

    The medical and surgical management of the chronic and recurrent esophageal and anal lesions of recessive dystrophic epidermolysis bullosa pose challenging problems for the physician. Various therapeutic approaches are discussed, and the case histories of four problem patients are reviewed.
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ranking = 1.4490033008087
keywords = epidermolysis bullosa, epidermolysis, bullosa
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4/10. Esophageal involvement in epidermolysis bullosa dystrophica.

    The clinical and radiographic findings in four cases of epidermolysis bullosa dystrophica of the esophagus are presented. The patients were 5-67 years old. All had typical skin lesions and dysphagia. The most impressive radiographic finding was bulla formation in virtually any part of the esophagus. The bullae would resolve or ulcerate. Scar formation with resultant stricture of the esophagus was a common sequela of this disease.
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ranking = 1.4490033008087
keywords = epidermolysis bullosa, epidermolysis, bullosa
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5/10. Esophageal involvement in epidermolysis bullosa dystrophica: clinical and roentgenographic manifestations.

    Epidermolysis bullosa is a rare hereditary mechanobullous skin disorder. Four patients with esophageal involvement are reported. These cases and review of reported cases of epidermolysis bullosa dystrophica-recessive (EBD-R) showed distinct clinical and roentgenographie features of esophageal involvement: diffuse inflammatory changes, motility disorders, small blisters or bullae seen as constant nodular-filling defects, esophageal ulcers, scars, pseudodiverticula, transverse and circumferential webs, various size strictures, shortening of the esophagus with development of traction hiatal hernia and gastroesophageal reflux, perforation, and complete obstruction of the esophageal lumen. Dysphagia is reversible when caused by bullae or webs and permanent when due to cicatrizing strictures. The strictures may remain unchanged in size for many years despite variations in dysphagic symptoms.
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ranking = 1.4649047191329
keywords = epidermolysis bullosa, epidermolysis, bullosa
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6/10. Esophageal web: a previously unrecognized complication of epidermolysis bullosa.

    The course of the esophageal disease in four patients with epidermolysis bullosa dystrophica recessive is examined. Three of four patients experienced web formation, a previously unrecognized finding, and the significance of this lesion in the evaluation and management of these patients is emphasized. One of four patients underwent a successful colonic interposition for severe esophageal disease and the role of this potentially life-saving procedure is discussed.
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ranking = 1.4490033008087
keywords = epidermolysis bullosa, epidermolysis, bullosa
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7/10. Acquired double-barrel oesophagus in epidermolysis bullosa dystrophica.

    An unusual case of epidermolysis bullosa dystrophica with extensive stenosis, high perforation, and dissection of the oesophagus forming a "double-barrel" structure is described. Gastric epithelium found in the upper oesophagus is thought to be of metaplastic origin and caused by repeated minor trauma with repair.
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ranking = 1.4490033008087
keywords = epidermolysis bullosa, epidermolysis, bullosa
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8/10. Oesophageal involvement in epidermolysis bullosa.

    epidermolysis bullosa dystrophica causing oesophageal stricture in two males of a family of 4 siblings--2 females and 2 males, and whose parents are not related, is reported. The stricture was treated with repeated dilatations and oral prednisolone on and off with complete success. A brief comment is also made with regards to the pathology and management of this condition.
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ranking = 1.1751040589711
keywords = epidermolysis bullosa, epidermolysis, bullosa
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9/10. Localized epidermolysis bullosa acquisita of the esophagus in a patient with Crohn's disease.

    epidermolysis bullosa acquisita is a rare autoimmune subepidermal bullous disease that affects both the skin and mucosae and is frequently associated with Crohn's disease. We report the case of a 27-yr-old man with Crohn's disease who presented with localized epidermolysis bullosa acquisita of the esophagus, without any other mucosal or cutaneous lesions. The patient was successfully treated with sulfasalazine.
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ranking = 4.3352528240326
keywords = epidermolysis bullosa acquisita, epidermolysis bullosa, bullosa acquisita, epidermolysis, acquisita, bullosa
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10/10. Nonscarring inflammatory epidermolysis bullosa acquisita with esophageal involvement and linear IgG deposits.

    A 24-year-old woman with autoimmune thrombocytopenia and hypothyroidism had an inflammatory bullous eruption in the mouth, face, and trunk that left no milia or scars after healing. Histologic examination revealed a subepidermal bulla and a neutrophil infiltration. Direct immunofluorescence examination showed deposition of IgG and C3 in the basement membrane zone (BMZ). Indirect immunofluorescence examination with 1M sodium chloride-split skin showed IgG binding to the dermal side. Immunoblot analysis demonstrated IgG autoantibodies reacting with 290 kD dermal protein. We diagnosed this as epidermolysis bullosa acquisita (EBA) with a nonscarring inflammatory feature. Treatment with oral dapsone, 75 mg, and prednisolone, 20 mg, cleared the eruption. Reduction of the prednisolone dosage was associated with multiple erosions in the esophagus. Direct immunofluorescence examination revealed linear deposition of IgG in the esophageal BMZ. To our knowledge, this is the first report of EBA with esophageal involvement and deposition of IgG in the BMZ of the esophagus.
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ranking = 4.193212956065
keywords = epidermolysis bullosa acquisita, epidermolysis bullosa, bullosa acquisita, epidermolysis, acquisita, bullosa
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