ABSTRACT
Hyperlipidemia of initially Type V and finally of Type III was observed in a patient with Weber-Christian disease. The lipoprotein lipase (LpL) and hepatic triglyceride lipase (HTGL) activities of the post-heparin plasma were low, but detectable. Both lipase activities were higher when assayed with 20 microliters of post-heparin plasma than with more than 30 microliters, indicating the presence of some inhibitory factor in the plasma. This plasma inhibited purified LpL and HTGL from human post-heparin plasma. Zonal ultracentrifugation studies showed that the inhibitor of hepatic lipase was associated with the middle fraction of intermediate lipoprotein and low density lipoproteins (1.020 less than d less than 1.040). These results are consistent with the idea that dyslipoproteinemia in this patient was partially due to dysfunction of the catabolic system caused with an inhibitor of lipolytic enzymes.
Subject(s)
Hyperlipidemias/etiology , Lipase/antagonists & inhibitors , Lipoprotein Lipase/antagonists & inhibitors , Panniculitis, Nodular Nonsuppurative/complications , Adult , Apolipoproteins/blood , Humans , Hyperlipidemias/enzymology , Male , Panniculitis, Nodular Nonsuppurative/blood , Panniculitis, Nodular Nonsuppurative/enzymologyABSTRACT
The diagnosis of Weber-Christian disease was made by clinical and histopathologic findings in a 25-year-old woman who had recurrent nodules on the legs and arms. The patient's history also disclosed multiple episodes of swelling trauma. Histopathologic examination of the lesions showed a prominent vasculitis. Studies of serum complement and kallikrein levels and of the fibrinolysis-clotting system showed a decrease in the levels of C3, C4, and total hemolytic complement activity and deficiencies (less than 20% of the normal values) of alpha 1-antitrypsin (alpha 1-AT) and antichymotrypsin activity. Chemical analyses of the patient's alpha 1-AT indicated a PiZZ genotype. Intermediate values of both inhibitor levels were detected in six family members. It is assumed that protease-inhibitor deficiencies predispose the development of panniculitis and vasculitis on trauma.
Subject(s)
Panniculitis, Nodular Nonsuppurative/enzymology , Protease Inhibitors/blood , Adult , Aprotinin/blood , Chymotrypsin/blood , Female , Humans , Kallikreins/blood , Panniculitis, Nodular Nonsuppurative/pathology , Phenotype , Skin/pathology , Trypsin/blood , alpha 1-Antitrypsin/analysisABSTRACT
Severe panniculitis of the Weber-Christian type occurred in two brothers, both with marked alpha 1-antitrypsin (alpha 1-AT) deficiency and phenotype PiZZ. Studies of inflammatory and immunologic function were undertaken in these two patients as well as in a third brother with severe alpha 1-AT deficiency but without Weber-Christian disease. The findings of these investigations were suggestive of exaggerated immunologic and inflammatory function with enhanced lymphocyte responsiveness to phytohemagglutinin, enhanced activation of neutrophils and monocytes, and accelerated delayed hypersensitivity responses in all three subjects. This hyperreactivity may explain the apparent association of alpha 1-AT deficiency with Weber-Christian disease.
Subject(s)
Panniculitis, Nodular Nonsuppurative/genetics , alpha 1-Antitrypsin Deficiency , Adult , Humans , Male , Monocytes/physiology , Neutrophils/physiology , Panniculitis, Nodular Nonsuppurative/enzymology , Panniculitis, Nodular Nonsuppurative/immunology , Phenotype , T-Lymphocytes/physiologyABSTRACT
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.
Subject(s)
Panniculitis, Nodular Nonsuppurative/enzymology , Peptide Hydrolases/blood , Adult , Afibrinogenemia/etiology , Factor VIII/immunology , Factor XIII , Female , Humans , Panniculitis, Nodular Nonsuppurative/complications , Thrombocytopenia/etiologyABSTRACT
A patient had an unusual acute, generalized panniculitis. The patient had a five-fold elevation of urinary amylase level and a slightly elevated serum lipase leval without any signs or symptoms of pancreatic disease. A secretin test caused an eightfold elevation in urinary amylase level and some elevation of serum lipase and amylase levels, whereas study of duodenal drainage revealed no abnormalities. Skin specimens from the lesions showed considerable amylase and lipase activity, whereas specimens from controls and from subsequent patients with panniculitis showed no such abnormalities. Autopsy showed a normal pancreas, both grossly and microscopically.
