PEDIATRICS Vol. 78 No. 1 July 1986, pp. 115-120
This Article
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Sorensen, R. U.
Right arrow Articles by Stern, R. C.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Sorensen, R. U.
Right arrow Articles by Stern, R. C.

Ten-Year Course of Early-Onset Weber-Christian Syndrome With Recurrent Pneumonia: A Suggestion for Pathogenesis

Ricardo U. Sorensen MD1, Carlos R. Abramowsky MD1, and Robert C. Stern MD1

1 From the Department of Pediatrics and Institute of Pathology, Case Western Reserve University School of Medicine, and Rainbow Babies and Childrens Hospital, Cleveland

A surviving 10-year-old boy with infant-onset systemic Weber-Christian syndrome is reported. He has had recurrent episodes of fever, aseptic panniculitis, and pneumonia. Although corticosteroid therapy has succeeded, colchicine and non-steroidal anti-inflammatory drugs have failed to abort or prevent acute episodes. The persistent leukocytosis (even during remission), the recurrent episodes of fever with associated increase in the acute phase reactants, and the failure of the nonsteroidal anti-inflammatory drugs leads us to propose that this form of Weber-Christian syndrome reflects an inborn error in the regulation of the inflammatory response. Systematic investigation of this hypothesis could yield important information on the normal regulation of inflammation and could lead to a rational therapeutic approach to this puzzling and usually devastating illness.

Key Words: Weber-Christian syndrome • panniculitis • pneumonia • inflammatory response

Submitted on June 28, 1985
Accepted on October 7, 1985