Generalized proteolysis in a young woman with Weber-Christian disease (nodular nonsuppurative panniculitis).
A patient with Weber-Christian disease (syn. nodular nonsuppurative panniculitis) is reported. The generalized cellular destruction in this patient resulted in liberation of proteolytic enzymes into the circulation, which led to multiple haemostatic disturbances with haemorrhagic diathesis. The most prominent haemostatic defects were thrombocytopenia with a normal life span of isologous platelets, high levels of AHF-related antigen, hypofibronigenaemia with short fibrinogen survival, low levels of Factor XIII (fibrin stabilizing factor = FSF) and increased amounts of fibrin/fibrinogen degradation products (FDP). Proteolytic enzymes, other than thrombin and plasmin which especially degrade Factor XIII and fibrongen, derived from destroyed cells (probably leukocytes) seem to have been involved in the pathogenesis of the bleeding disorder in this patient.
D000328:Adult; D000347:Afibrinogenemia / Q000209:etiology; D005169:Factor VIII / Q000276:immunology; D005176:Factor XIII; D005260:Female; D006801:Humans; D010201:Panniculitis, Nodular Nonsuppurative / Q000150:complications / Q000201:enzymology*; D010447:Peptide Hydrolases / Q000097:blood*; D013921:Thrombocytopenia / Q000209:etiology
D005169:Factor VIII; D005176:Factor XIII; D010447:Peptide Hydrolases