title: Ten-year course of early-onset Weber-Christian syndrome with recurrent pneumonia: a suggestion for pathogenesis.
abstract: 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.
mesh_terms: D002648:Child; D003131:Combined Modality Therapy; D006801:Humans; D007964:Leukocytosis / Q000150:complications; D008297:Male; D010201:Panniculitis, Nodular Nonsuppurative / Q000150:complications* / Q000151:congenital / Q000628:therapy; D011014:Pneumonia / Q000150:complications* / Q000000981:diagnostic imaging; D011859:Radiography; D012867:Skin / Q000473:pathology