Ehlers-Danlos syndrome associated with acute pancreatitis.
The Ehlers-Danlos syndrome is a polysystemic inherited connective tissue disease characterized by articular laxity, hyperelastic skin, a tendency to excessive bleeding in the presence of minimal trauma, and friability of different tissues. The syndrome is genetically, biochemically and clinically heterogeneous and several well defined subtypes have been identified. We describe a patient with the type II variant of this syndrome who developed acute spontaneous pancreatitis. One brother, among a total of 8 affected siblings in the family, also had symptoms of acute spontaneous pancreatitis. The association of acute spontaneous pancreatitis with the Ehlers-Danlos syndrome has not been previously reported.
Sarra-Carbonell
S
S
Department of Medicine, University of Chile, Santiago.
Jimenez
S A
SA
D000208:Acute Disease; D000293:Adolescent; D002767:Cholecystostomy; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000235:genetics / Q000503:physiopathology; D005260:Female; D006801:Humans; D010195:Pancreatitis / Q000150:complications* / Q000601:surgery; D010375:Pedigree