Epidermolysis bullosa: radiographic findings in 16 cases

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

“…This frequency increases with age, with a cumulative risk of almost 95% by 45 years of age (Fig 6). 44,45,51,52 Progressive scarring and narrowing of the esophagus limit the food textures that can be ingested, presenting initially with dysphagia to hard or bulky foods, then to softer foods, and, eventually, to liquids. If left untreated, patients ultimately may be unable to swallow even their own saliva.…”

“…The majority of strictures occurs in the upper third of the esophagus, but may arise anywhere else along its length. 53-55 It [41][42][43][44][45]51,[53][54][55]64,67,68,158,159 Esophageal web 54,56,57 Spontaneous esophageal perforation 72,73 Vomiting of an esophageal cast 42,50 Gastroesophageal reflux disease 43-45,47,z Abnormal esophageal motility 43,71 Hiatus hernia 44 Gastritis/peptic ulcer disease 44 Pyloric atresia or stenosis 86 has been suggested that upper esophageal strictures might arise from blistering related to the ingestion of food, causing direct damage to the esophageal mucosa, whereas lower strictures may be precipitated or exacerbated by GERD. Strictures in EB may be solitary or, in approximately 10% to 40% of patients, multiple, and can be demonstrated radiologically with contrast studies.…”

Absceso paraesofágico: una causa poco frecuente de disfagia en la epidermólisis ampollosa

“…Con esta sospecha, se realizó un esofagograma que confirmó la presencia de una estenosis casi completa de la luz esofágica en el tercio superior. La afectación esofágica de la epidermóli-sis ampollosa tiene imágenes características en los estudios baritados consistentes en estenosis largas, de bordes suaves y afiladas en su extremo distal, aunque también pueden verse estenosis segmentarias o membranas 6,7 . La formación de seudodivertículos, dilataciones preestenóticas, ulceraciones y espasmos se incluyen dentro de los hallazgos menos frecuentes.…”

Fluoroscopically Guided Endoluminal Balloon Dilatation of Esophageal Stricture Due to Epidermolysis Bullosa Dystrophica

“…The disease can be acquired or inherited as an autosomal dominant or recessive trait and is further subclassi®ed by the layer of the skin involved in the separation [3].…”

“…The most common extracutaneous site of involvement in RDEB is the gastrointestinal tract. The esophagus and the oropharynx are the most frequently involved sites of the gastrointestinal tract [1,3]. Gastrointestinal complications are a major cause of morbidity, nutritional de®cien-cies, and growth retardation seen in RDEB [1].…”