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1/10. 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.
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ranking = 1
keywords = nonsuppurative
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2/10. Weber-Christian disease after weight loss.

    Weber-Christian disease has no known cause and is characterized by a febrile, recurrent, nonsuppurative panniculitis occurring preferentially in young, obese women. The present case is of interest in that it occurred in a young, obese woman after she lost 18.2 kg (40 lb) in eight weeks. It is only speculative as to whether the sudden weight loss precipitated the Weber-Christian disease. However, since Weber-Christian disease is an obscure disease with metabolic and autoimmune overtones, such a hypothesis is not unrealistic in a predisposed individual.
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ranking = 1
keywords = nonsuppurative
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3/10. Suppressor-cytotoxic T-lymphocyte panniculitis.

    A 3 1/2-year-old girl had fever and nonsuppurative panniculitis. Biopsies revealed lobular lymphocytic panniculitis. An extensive evaluation of the patient for infectious and other systemic diseases yielded negative results. Although the number of peripheral T lymphocytes was normal, suppressor-cytotoxic (OKT8) lymphocytes predominated in the subcutis of the lesions. Suppressor-cytotoxic T-lymphocyte panniculitis may represent a unique type of panniculitis.
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ranking = 1
keywords = nonsuppurative
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4/10. A case of Weber-Christian disease accompanied by a nasal symptom (a clinicopathologic case report).

    A rare case of fatal Weber-Christian disease is reported. The initial presenting signs were unilateral nasal obstruction due to hyperplasia of the ulcerated turbinate, swollen cheeks and eyelids accompanied by recurrent febrile episodes. The nasal lesion showed marked inflammatory granulation tissue with necrosis and there were 'atypical' reticulohistiocytic cells. Subsequently the patient developed widespread nodular nonsuppurative panniculitis of the left thigh. The patient's general condition worsened in spite of cytotoxic and steroid treatment and he died of acute peritonitis from a perforated duodenal ulcer complicating the treatment. microscopy showed that some atypical cells were present not only in the nasal lesion but also in the lesions of the skin and in the sinusoids of the liver and in the spleen. Weber-Christian disease secondary to midline granuloma or malignant histiocytosis has not previously been reported.
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ranking = 1
keywords = nonsuppurative
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5/10. Nodular nonsuppurative panniculitis associated with jejunoileal bypass surgery.

    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.
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ranking = 6
keywords = nonsuppurative
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6/10. amyloidosis (AA type) associated with nodular nonsuppurative panniculitis.

    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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ranking = 5
keywords = nonsuppurative
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7/10. Xanthogranuloma of the dura in systemic Weber-Christian disease.

    A 51-year-old woman who had systemic Weber-Christian disease (WCD) displayed multiple xanthomatous lesions and a large xanthogranuloma of the tentorium cerebelli with signs of brainstem and cerebellar compression. Three other cases of dural xanthomatous lesions associated with systemic WCD have been reported. These dural and leptomeningeal xanthomatous lesions in WCD, a nonsuppurative inflammatory condition, are similar to those of hand-Schuller-Christian disease, a quasi-neoplastic process.
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ranking = 1
keywords = nonsuppurative
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8/10. chloroquine-induced remission of nodular panniculitis present for 15 years.

    A 62-year-old woman experienced a continuous series of subcutaneous masses of the arms, buttocks, and legs which had resisted treatment for over 15 years. On the arms and buttocks the lesions were nonerythematous and asymptomatic, but those on the lower leg were at times tender and erythematous. The nodules, which proved on biopsy to be nodular, nonsuppurative panniculitis, slowly receded without residual skin changes as new ones appeared. Bacterial hypersensitivity may have played a role in their pathogenesis since fibrin microclot induction tests showed a marked sensitivity to gram-negative bacteria. Although there was never any clinical, serologic or histologic evidence of lupus, the lesions completely disappeared as a result of long-term low-dose chloroquine treatment.
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ranking = 1
keywords = nonsuppurative
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9/10. Nodular nonsuppurative panniculitis in association with primary biliary cirrhosis and Hashimoto's thyroiditis.

    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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ranking = 7
keywords = nonsuppurative
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10/10. Acute panniculitis: a clinical and histopathologic study of 34 cases.

    Thirty-four patients had histopathologic findings of acute panniculitis. 15 had clinical findings of erythema nodosum; 6 had infectious lesions; and 5 had Weber-Christian-like conditions with recurrent febrile suppurative or nonsuppurative nodular eruptions; 3 of the 5 had amylase and lipase enzyme abnormalities with or without pancreatic disease. Five additional patients had acute panniculitis that would be termed "deythema induratum" or "nodular vasculitis" by most clinicians, and the subsequent pathologic findings and courses were consistent with this view. Two patients had limited, traumatic panniculitis, forming a distinctive group, but trauma was a factor in lesion formation in all groups, and one patient had vasculitis. Whereas acute panniculitis, with or without fat necrosis, may be associated with many inflammatory syndromes of the skin and subcutaneous tissue and may rarely occur in all, the most common relationship is with erythema nodosum in its most acute forms.
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ranking = 1
keywords = nonsuppurative
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