Epidermolysis bullosa
School ager
with dysphagia. AP (left) and lateral (right) images from an esophagram show a persistent long narrowing in the mid esophagus.The diagnosis was an esophageal stricture due to epidermolysis bullosa.
Epidermolysis
bullosa • Epidermolysis bullosa (hands) - Ganzer Fall bei Radiopaedia
Death from
colonic disease in epidermolysis bullosa dystrophica. (A) Computed tomography (CT) of abdomen showed extra-luminal free air in the peritoneal cavity (white arrow), and marked distension of colonic loop with fecal impaction (black arrow). (B) Fecal impaction with marked colon distention was noted in the distal sigmoid colon (black arrow).
Epidermolysis bullosa refers to a rare group of genetically determined conditions characterized by blistering of the skin. This can be limited to the soles and palms or extensive whole body involvement.
Radiographic features
Gastrointestinal manifestations:
- limited to the mucosa of the gastrointestinal tract, and predominantly the esophagus with the formation of submucosal bullae acutely and chronically esophageal webs
Urogenital manifestations :
- scarring of the penis glans or the vaginal vestibule with associated urethral strictures can lead to urinary outflow tract obstruct and consequent hydroureter and hydronephrosis
Musculoskeletal manifestations :
- blistering and scarring may also cause fusion of fingers and toes
- contractures involving multiple joints
- muscular dystrophy
Siehe auch:
Assoziationen und Differentialdiagnosen zu Epidermolysis bullosa: