[Nonsuppurative nodular panniculitis. Pfeifer-Weber-Christian syndrome].
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A 32-year-old woman underwent jejunoileal bypass surgery for morbid obesity. Her postoperative course was marked with many of the complications of this type of surgery. In addition, tender, erythematous nodules developed on her legs and abdomen that were grossly and microscopically consistent with nodular nonsuppurative panniculitis. She did not have any condition or disease previously reported to be associated with nodular nonsuppurative inflammation. The nodular panniculitis in this patient appears to be a new complication of small-bowel bypass surgery.
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.
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A 42-year-old man had a 9-year history of idiopathic, nodular nonsuppurative panniculitis that was complicated by generalized AA-type amyloidosis. We believe that this is the first report of this association.
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Recurrent episodes of livid, painful, subcutaneous nodules on both lower extremities with consecutive soft tissue atrophy developed in a 57-year-old woman with previously undiagnosed primary biliary cirrhosis (PBC) and Hashimoto's thyroiditis. Histological examination of a biopsy taken from an active area of the skin showed nodular nonsuppurative panniculitis. Immunosuppressive therapy with prednisone was necessary to control disease activity. The etiology and pathogenesis of nodular nonsuppurative panniculitis is still unknown. Frequently, the disease occurs in patients with autoimmune disorders. The association with PBC and Hashimoto's disease as described herein reinforces the view that nodular nonsuppurative panniculitis may be the response of the subcutaneous adipose tissue to an unknown autoimmune stimulus.
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