Systemic Weber-Christian Disease Research Articles

Childhood Weber–Christian disease: clinical investigation and virus detection

To investigate clinical features of childhood Weber-Christian disease (WCD) and the relationship between WCD and virus. The clinical characteristics, laboratory findings, therapy and clinical course of nine patients were reviewed and the viral antigens (EBV, CMV, HSV1 and HSV2) were detected. They were six males and three females with a median age of 9 years. They included four simple and five systemic WCD, and all presented fever and tender subcutaneous nodules. Other clinical features included hepatomegaly in nine patients, splenomegaly in two, lymphadenhypertrophy in six, respiratory system involved in two, gastrointestinal tract involved in two and convulsion and bleeding in one. Anaemia was noted in seven patients, raised ALT in three, hypergammaglobulnaemia in three, proteinuria in one and three of six patients positive of antinuclear antibodies (ANA). No viral antigens were found. Corticosteroids therapy was administrated for all patients, immunoglobulin for two, non-steroidal anti-inflammatory agents for three and cyclophosphamide for one. Five patients released after therapy and three patients died. WCD is a severe disease characterized by relapsing, febrile subcutaneous nodules and biopsy is needed for early diagnosis. The viral infection may not be associated with WCD.

ＳＬＥにｓｙｓｔｅｍｉｃ Ｗｅｂｅｒ Ｃｈｒｉｓｔｉａｎ病を伴いタクロリムスが著効を示した１例

We report a 30 years old systemic lupus erythematosus (SLE) female patient, who had been diagnosed with systemic Weber Christian disease, presented with acute onset of high fever, hepatomegaly, lower leg edema with multiple subcutaneous nodules. Laboratory tests showed elevated CRP, fibrinogen and liver enzymes, especially LDH and LAP, with coagulation abnormalities. There is a marked enlarged fatty liver in both abdomen echo and CT and chest X-ray films showed pleural effusion in both sides, without pulmonary lesions. However, complements, ANA and anti-DNA antibody etc concerning activity of SLE didn't moved. A diagnosis of systemic Weber Christian disease was made based on clinical and laboratory examinations and histopathological findings. The therapy of standard treatment, such as steroid, plus pulse steroid therapy was performed. The response, however, is unpredictable. After this, tacrolimus hydrate was administed intravenously in an initial dose of 1.2 mg/body and in oral maintenance of 1.0 mg/ body with a drastic response and patient completely recovered within 1 month. To our knowledge, this is the first report of SLE complicated systemic Weber Christian disease and is the first report of successful treatment of systemic Weber Christian disease with tacrolimus hydrate.

Lupus erythematosus panniculitis of the breast

A case of a patient presenting disseminated lupus erythematosus (DLE) with panniculitis located in the breast is described. The mammographic appearance of the calcifications found in this case is highly suggestive of panniculitis. It was previously described with other systemic diseases and Weber-Christian disease, but not with LE panniculitis.

An Intracranial Mass Lesion in Systemic Xanthogranulomatosis: Case Report

Abstract The authors describe a 42-year-old woman with systemic xanthogranulomatosis and bilateral intraorbital tumors, who subsequently developed multiple lesions of the intracranial dura mater, spinal cord, retroperitoneum, pericardium, and mediastinum. Systemic xanthogranulomatosis is histologically similar to systemic Weber-Christian disease, except for the absence of subcutaneous panniculitis. Immunohistochemical studies suggest that this clinical entity can be differentiated from histiocytosis X, because foamy cells in systemic xanthogranulomatosis demonstrate macrophages but not T-zone histiocyte markers. Differentiation of this disease from other intracranial xanthogranulomas and treatment are discussed.

An intracranial mass lesion in systemic xanthogranulomatosis

The authors describe a 42-year-old woman with systemic xanthogranulomatosis and bilateral intraorbital tumors, who subsequently developed multiple lesions of the intracranial dura mater, spinal cord, retroperitoneum, pericardium, and mediastinum. Systemic xanthogranulomatosis is histologically similar to systemic Weber-Christian disease, except for the absence of subcutaneous panniculitis. Immunohistochemical studies suggest that this clinical entity can be differentiated from histiocytosis X, because foamy cells in systemic xanthogranulomatosis demonstrate macrophages but not T-zone histiocyte markers. Differentiation of this disease from other intracranial xanthogranulomas and treatment are discussed.

Multiple Intracranial Xanthogranulomas

A 58-year-old female was admitted to our hospital with nausea, vomiting, and gait disturbance of 1 year duration. Postcontrast computed tomographic scans demonstrated enhanced lesions in the left cerebellopontine angle (CPA), the retrosellar region, the right parasellar region, and the left parietooccipital convexity. The left parieto-occipital tumor was totally removed in the first operation and the left CPA tumor was partially removed in the second. The histological diagnosis of both tumors was xanthogranuloma. She also had cutaneous lesions (subcutaneous nodules without tenderness) and an ureteral stenosis possibly due to the retroperitoneal involvement. A skin biopsy demonstrated infiltration of xanthoma cells and foamy cells in the dermis. A gallium scintigram demonstrated an abnormal uptake in the thoracic cavity, liver, and bones. From these findings, systemic Weber-Christian disease was diagnosed. Another unique aspect was that the serum IgE levels were increased during postoperative hospitalization. This suggests that abnormal immunological conditions are related to this disease and that intracranial xanthogranulomas are a manifestation of systemic Weber-Christian disease.

Systemic Weber–Christian Disease with Portal Hypertension and Oesophageal Varices

Systemic Weber–Christian Disease with Portal Hypertension and Oesophageal Varices

Sterile splenic abscesses in systemic Weber-Christian disease: Unique source of abdominal pain

A patient with Weber-Christian disease and relapsing abdominal pain due to sterile splenic abscesses is presented. Histologically, acute and chronic inflammation with focal suppurative infiltrates of phagocytosed fat was detected in the absence of vascular or embolic disease, infection, or other apparent cause. Abdominal discomfort, an uncommon manifestation of Weber-Christian disease, usually is related to mesenteric panniculitis. Sterile splenic abscesses represent a previously undescribed complication of Weber-Christian disease, and another source of abdominal pain in this disorder. Although the precise pathophysiology of sterile splenic abscess formation in Weber-Christian disease is unknown, splenectomy is an effective means of alleviating pain.

Possible immune basis for abnormal fat metabolism in systemic Weber-Christian disease

Possible immune basis for abnormal fat metabolism in systemic Weber-Christian disease

Systemic form of Weber-Christian disease

A case of systemic Weber-Christian disease, involving retroperitoneal adipose tissue with secondary damage to the associated organs and blood vessels is reported. The involved organs showed varying degrees of necrosis, lipogranulomata and fibrosis.

Systemic Weber-Christian Disease Complicated with Pancytopenia and Megaloblastosis: Report of a Case with Autopsy

Systemic Weber-Christian Disease Complicated with Pancytopenia and Megaloblastosis: Report of a Case with Autopsy

Systemic idiopathic fibrosis and systemic Weber-Christian disease

Idiopathic retroperitoneal fibrosis is represented as one of the manifestations of a widespread disease, systemic idiopathic fibrosis. Some evidence is presented which suggests its origin as an inflammatory disease of adipose tissue. Although relatively rare, the idiopathic diseases of adipose tissue and its blood vessels are worthy of closer study.

SYSTEMIC WEBER-CHRISTIAN DISEASE.

A patient suffering from Weber-Christian panniculitis was found at necropsy to have similar lesions in the visceral adipose tissue. When the lesions occur internally, diagnosis during life is complicated by the difficulty of examining visceral adipose tissue and the disease may often go unrecognized, but patients suffering from Weber-Christian panniculitis should be investigated bearing in mind the possibility of internal lesions. Only 11 cases confirmed by necropsy appear to have been reported hitherto. The aetiology remains obscure. It is suggested that the name ;systemic Weber-Christian disease' be applied when the lesions occur in adipose tissue other than the panniculus adiposus and that the term ;Weber-Christian panniculitis' be used when the lesions are confined to the subcutaneous adipose tissue.