Sclerosing mesenteritis. Response to cyclophosphamide.

We present two patients with an aggressive form of sclerosing mesenteritis characterized by a progressive, life-threatening course, prominent retroperitoneal disease, and tubuloreticular structures in one case, an ultrastructural feature associated with autoimmune and cyclophosphamide-responsive diseases. In both patients, aggressive immunosuppressive medical therapy with cyclophosphamide resulted in prompt, dramatic improvement, without recurrence. When the diagnosis of sclerosing mesenteritis is established, we recommend early aggressive medical therapy with cyclophosphamide, particularly when tubuloreticular structures are present.

Chronic nodular panniculitis in a three-year-old mare.

The clinical signs of chronic nodular panniculitis in a three-year-old mare were consistent with a diagnosis of sterile nodular panniculitis, but the mare had histopathological signs of both generalised steatitis and sterile nodular panniculitis. The mare was deficient in vitamin E and selenium.

Subarachnoid hemorrhage of unknown origin associated with Weber-Christian disease--case report.

A 58-year-old female, who had suffered from Weber-Christian disease for 26 years, presented with subarachnoid hemorrhage. Cerebral angiography showed dilatation of the basilar tip. An operation was performed in the chronic stage. The wall of dilated basilar artery was tough, but that of the right superior cerebellar artery was very thin and three small aneurysms were found on the right middle cerebral artery. Blood levels of fibrin degradation products, plasmin-alpha 2-plasmin inhibitor complex, and thrombin-antithrombin III complex were increased. The abnormality of the coagulation-fibrinolysis system and the fragility of the cerebral arteries related to Weber-Christian disease were probably the cause of the subarachnoid hemorrhage.

[Weber-Christian syndrome and pancreatic involvement. Apropos of a case with review of the literature]. / Syndrome de Weber-Christian et affections pancréatiques. A propos d'un cas avec revue de la littérature.

The Weber-Christian syndrome associates generalized nodular inflammatory panniculitis with osteoarticular manifestations. Pathogenesis of this rare affection is still obscure but a pancreatic origin has been reported in the literature. A case is presented having the particular features of being revealed by a subacute episode of chronic pancreatitis, and by the intensity of radiologic bony manifestations.

A case of Weber-Christian disease with later development of rheumatoid arthritis.

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.

[Pfeifer-Weber-Christian syndrome of the mesentery. Clinical and pathologico-anatomical aspects]. / Pfeifer-Weber-Christian-Syndrom des Mesenteriums. Klinische und pathologisch-anatomische Aspekte.

A panniculitis isolated solely to the fat tissue of the mesentery is rarely encountered. Problems related to the diagnosis, therapy, and-patho-anatomical features of this polyetiological disease are presented. Based on the excellent response to antirheumatic therapy accompanied by a positive HLA B27, this case could be classified as a connective tissue disease.

[Mesenteric panniculitis]. / Paniculitis mesentérica

A 4-year-old girl was studied at the Pediatrics Unit at the Hospital General de México, S.S.A., with a diagnosis of abdominal tumor, probably malignant. After several laboratory tests (blood count, blood chemistry, catecholamines and the following X-ray studies: chest, skull, long bones, Cava's phlebography and excretory urography), she was submitted to surgery without a diagnosis. After this, the final diagnosis was established: mesenteric panniculitis (mesenteric lipodystrophy. This case is published because of its rare occurrence at this age and because it is important to be kept in mind in the differential diagnosis of abdominal tumors in pediatric practice.

Hepatic Weber-Christian disease.

Weber-Christian disease is an idiopathic disorder characterized by nonsuppurative nodular panniculitis with a lobular distribution of acute inflammation in the subcutaneous fat with occasional systemic involvement. Although the histopathologic features of the liver disease in the syndrome are characterized by steatohepatitis, the clinical features have not been well defined. We report a case of hepatic Weber-Christian disease and discuss the clinical differences from steatohepatitis due to the more common disorders of obesity and diabetes mellitus.

Nonsuppurative nodular panniculitis (Weber-Christian disease). An unusual cause of mammary calcifications.

A case of nodular panniculitis (Weber-Christian disease) with breast involvement is presented. Xeromammography demonstrated unusual calcifications in the nodules.

CT features of mesenteric panniculitis.

Mesenteric panniculitis (MP) is an inflammatory process of the mesenteric fat associated with a variable amount of fibrosis. We present the CT findings in four patients with MP. In three patients CT showed a fatty mass in the root of the mesentery and surrounding the mesenteric vessels without distorting them. The fourth patient presented an unusual form of MP with radiologically visible calcifications. Diagnosis was suggested preoperatively in two cases.

Mesenteric panniculitis.

Mesenteric panniculitis is an inflammatory condition of mesenteric fat which may result in peritoneal space occupying lesions. Only two previously reported cases in the English literature have been evaluated by computed tomography (CT). We present 2 additional cases and emphasize that CT patterns are not specific, but suggest the diagnosis in the presence of a well-defined fibrous wall circumscribing a fatty mass containing regions with attenuation near that of water. Lymphoma and mesenteric metastases should be considered the most frequent radiologic, clinical and surgical differential diagnosis.

Acinar pancreatic tumor with metastatic fat necrosis: report of a case and review of rheumatic manifestations.

This report deals with a pancreatic tumor associated with metastatic fat necrosis. Our patient displayed the full gamut of nodular panniculitis, polyarthritis, fever, eosinophilia, hyperlipasemia, lytic bones lesions, and marrow fat necrosis. The rheumatologic features are reviewed. Elevated serum lipase is a most helpful laboratory confirmation. The tumor in our patient presented a difficult problem in classification. Although the appearance under light microscopy was most compatible with islet cell carcinoma or islet cell carcinoid, the ultrastructural characteristics were those of acinar carcinoma.