A case of Weber-Christian disease with later development of rheumatoid arthritis.
(9/24)
Weber-Christian disease (WCD) is a syndrome characterized by recurrent subcutaneous nodules, fever, occasional lipoatrophy, fatigue, arthralgia, and myalgia. We report a case of WCD associated with rheumatoid arthritis. A 65-year-old woman consulted our outpatient clinic because of bilateral hand swelling. The patient had presented with fever and subcutaneous nodules in her trunk and upper and lower extremities in 1983. At that time, the dermatology department diagnosed this patient as having WCD after biopsy of the nodules demonstrated lobular panniculitis. She has been treated with corticosteroid (5-15 mg/day) since then. The patient continued to have recurrent episodes of transient inflammatory arthritis in the small joints of the fingers and fever, and was initially assessed at our institution in October 2007. Finally, in November 2007, she was diagnosed as having both WCD and rheumatoid arthritis (RA) and treated with corticosteroid (5 mg/day) and methotrexate (MTX) (7.5 mg/week). Thereafter, her clinical symptoms gradually improved. This is the second case of WCD showing the subsequent development of RA, successfully treated with MTX, in the English literature. This case may provide clinical insight into WCD and RA. (+info)
Weber-Christian disease developing into mediastinitis and pleuritis with massive pleural effusion.
(10/24)
A 53-year-old man visited our hospital complaining of high fever. Chest computed tomography showed left pleural effusion and mediastinitis. He developed painful red subcutaneous nodules in his bilateral lower extremities. Thoracoscopy-assisted exploratory excision showed visceral pleura thickening; panniculitis in the periaortic area was histologically proven. The patient was treated with corticosteroid therapy which immediately reduced the fever. Subsequent imaging examinations after corticosteroid therapy showed improvement of mediastinitis and pleural effusion. This case reminds us that Weber-Christian disease (WCD) should be included in the differential diagnosis of mediastinitis although WCD is rarely associated with thoracic involvement. (+info)
Weber-Christian disease producing splenic vein occlusion and bleeding gastric varices: successful treatment with sclerotherapy.
(11/24)
A 48 year old woman with intra-abdominal Weber-Christian disease presented with bleeding gastric varices and evidence of splenic vein occlusion. We describe the problems encountered in making this diagnosis and subsequent treatment. (+info)
Systemic Weber-Christian disease with reversible bilateral ureteric obstruction.
(12/24)
It has been proposed that idiopathic retroperitoneal fibrosis may be a consequence of 'healed' retroperitoneal lesions of systemic Weber-Christian disease. However ureteric obstruction which is the hallmark of idiopathic retroperitoneal fibrosis, has not been described in systemic Weber-Christian disease. We report a patient with systemic Weber-Christian disease who, during a relapse, developed bilateral ureteric obstruction which resolved when the Weber-Christian disease remitted. The radiological appearances were consistent with a diagnosis of idiopathic retroperitoneal fibrosis, but the clinical course was slightly atypical in that the ureteric obstruction resolved completely and rapidly. Ureteric obstruction can complicate systemic Weber-Christian disease and this observation gives support to the hypothesis that idiopathic retroperitoneal fibrosis is related to systemic Weber-Christian disease. (+info)
Dyslipoproteinemia and an inhibitor of lipolytic enzymes in Weber-Christian disease.
(13/24)
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. (+info)
Weber-Christian panniculitis with immune complex glomerulonephritis.
(14/24)
The etiology of Weber-Christian panniculitis is unknown. A case is presented in which an association is found between immune complex glomerulonephritis and a relapse of Weber-Christian panniculitis. This case adds to the literature, implicating immune system dysfunction in the pathophysiology of this disease. (+info)
Weber-Christian panniculitis.
(15/24)
Two cases of Weber-Christian panniculitis with onset at 7 months and 3 years 6 months are described. Both show evidence of disturbances in immune mechanisms. The family of the first case has a high prevalence of coeliac disease, and the mother of the first patient also suffers from alopecia areata and dermatitis herpetiformis. (+info)
Systemic nodular panniculitis with cardiac involvement.
(16/24)
A case of systemic nodular panniculitis is described in which the myocardium was found at necropsy to be extensively involved with focal interstitial carditis, identical histologically with nodules of panniculitis biopsied from the skin. This degree of myocardial involvement, which was not apparent during life and was not confined to pericardial or myocardial adipose tissue, has not previously been reported. The literature relating to nodular panniculitis is briefly reviewed and the concept of Weber-Christian disease critically re-appraised. (+info)