[Familial paroxysmal polyserositis. Previously unpublished peritoneal complications. A case].
A young unmarried Lebanese woman presenting with periodic disease (familial paroxysmal polyserositis) since she was 3 months old developed recurrent abundant ascites at the age of 21 years. Several hundred millilitres of strongly eosinophilic fluid were evacuated. Exploratory laparotomy unexpectedly disclosed an encapsulating peritonitis with adhesions involving the small bowel and the ascending colon; there were masses of lipid-laden cells, clusters of cholesterol/crystals and marked mesoepithelial reaction. In view of the patient's dramatic response to colchicine 2 mg/day, these findings were regarded as being related to the periodic disease.
Bitar
E
E
Rizk
A
A
Nasr
W
W
Gédéon
E M
EM
Tabbara
W
W
D001201:Ascites / Q000209:etiology / Q000473:pathology; D003078:Colchicine / Q000627:therapeutic use; D010505:Familial Mediterranean Fever / Q000150:complications / Q000188:drug therapy / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007861:Lebanon; D010532:Peritoneal Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D010537:Peritoneum / Q000473:pathology
D003078:Colchicine
Polysérite paroxystique familiale. Complications péritonéales inédites. Une observation.