Epidermolysis Bullosa of the Esophagus: Report of Two Cases and Review of Literature

The Gut in Skin Disease: Gastro-Intestinal Manifestations of Selected Skin Disorders

“…Dystrophic EB ( fig. 2) is a severely mutilating form of the disease affect ing young children in whom almost constant oropharyngeal ulceration impairs eating, and insidious dysphagia caused by bullae and subsequent stenoses, usually at the upper and lower ends of the oesophagus, further com promises the nutritional state [24], The con dition may also involve the anus, and epi sodic diarrhoea may occur synchronously with exacerbation of the cutaneous lesions [25], Junctional epidermolysis bullosa (EB letalis) and its non-lethal variant, generalised atrophic benign EB, cause intestinal involve ment much less commonly. Acquired EB is a term applied to blistering in adult life without evidence of familial transmission.…”

Esophageal involvement in epidermolysis bullosa dystrophica: Clinical and roentgenographic manifestations

“…Dysphagia usually starts insidiously in the first decade of life, but delay until the fifth decade has been reported. Fifty percent of the patients reviewed by Nix and Christianson [7] showed dysphagia for more than 20 years. Three out of 4 of the patients reported here had symptoms of dysphagia for at least I0 years.…”

“…Three out of 4 of the patients reported here had symptoms of dysphagia for at least I0 years. The esophageal lesions that start by adolescence may contribute to patient's demise through inanition or aspiration [7,14]. Disordered Motility.…”

“…Complete occlusion of the lumen of the esophagus may occur due to diffuse bullous and ulcerative lesions of the esophagus, stenotic lesions 2-6 cm in length, and multiple areas of stricture formation. A markedly narrowed stricture may trap food particles and lead to complete luminal occlusion [7]. Case 3 developed esophageal occlusion due to diffuse bullous disease during an episode of bullous eruptions.…”

“…Nix et al [7] reported a 22-year-old man with EBD-R since birth and esophageal lesions since 8 years of age, who perforated his esophagus while attempting to eat a toasted cheese sandwich. A stricture trapped food particles causing obstruction, which prevented adequate propulsion of the esophageal contents.…”