Epidermolysis and Pemphigoid

Published on 19/07/2015 by admin

Filed under Radiology

Last modified 22/04/2025

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 Most commonly in upper 1/3 of esophagus

• Esophageal webs seen early in benign mucous membrane pemphigoid; occasionally seen in epidermolysis bullosa

CLINICAL ISSUES

• Both diseases are rare
• Epidermolysis bullosa

image Presents in early childhood
image Severe skin blistering, nail dystrophy
image Webbing between fingers and toes, can eventually lead to contractures or amputation of digits
image Diffuse osteopenia, muscle atrophy secondary to underuse
image Genitourinary abnormalities: Vaginal stenosis, bladder wall thickening
• Benign mucous membrane pemphigoid

image Presents in 4th decade
image Bullae, preference for mucous membranes
image Individual bullae may resolve or progress to ulceration or stenosis
image Oral mucosa involved in all patients, conjunctiva in most patients
• Treatment: Endoscopic dilation may be tried but it may lead to worse strictures
• Colonic interposition: To bypass refractory esophageal lesions
image
(Left) Oblique esophagram in a 19-year-old man with epidermolysis bullosa shows a tight, long stricture image of the proximal esophagus. The proximal end of the stricture is fairly abrupt, while the distal end is more tapered.

image
(Right) Oblique esophagram in the same patient after attempted dilation shows that the stricture remains image, now with extravasation of the contrast material image, indicating perforation. The contrast material extends along the esophageal wall within the mediastinum.
image
(Left) Spot film from an esophagram in a 30-year-old woman with severe epidermolysis bullosa demonstrates a long stricture image of the distal esophagus. Other films demonstrated more proximal strictures. These were treated with balloon dilation but have recurred.

image
(Right) Lateral esophagram in an 18-year-old man shows a web-like stricture image near the pharyngoesophageal junction that is the result of repeated episodes of mucosal ulceration, typical of patients with epidermolysis bullosa.