George E. Ehrlich, MD
Ehrlich GE. Genetics of Familial Mediterranean Fever and Its Implications. Ann Intern Med. 1998;129:581-582. doi: 10.7326/0003-4819-129-7-199810010-00014
Download citation file:
Published: Ann Intern Med. 1998;129(7):581-582.
In 1945, Dr. Sheppard Siegal, an allergist at Mount Sinai Hospital in New York, reported in Annals five cases of “recurrent paroxysms of severe abdominal pain with fever … as high as 105 °F,” all in men and all starting early in life. He named the syndrome benign paroxysmal peritonitis . The peritoneal signs were so severe that “emergency operation [had] been repeatedly urged.” Pleuritic chest pain often accompanied the attacks. At least one of the patients had “urticarial” lesions near the ankle, and another had intermittent joint pains, usually monoarticular. As an allergist, Siegal approached these cases from his own vantage point, and the history of this condition is replete with references to potential allergens and allergic relatives. Unfortunately, except for noting that all of the patients were white men, he provided no other ethnic background data. Although an occasional similar case was found in previous published series, including some in which such manifestations were interpreted as “Henoch” purpura (from which Siegal clearly differentiated the condition), this landmark article was essentially the first to describe the clinical presentation of what is now known as familial Mediterranean fever.
to gain full access to the content and tools.
Learn more about subscription options.
Register Now for a free account.
Results provided by:
Copyright © 2016 American College of Physicians. All Rights Reserved.
Print ISSN: 0003-4819 | Online ISSN: 1539-3704
Conditions of Use
This PDF is available to Subscribers Only