1/134. Cytophagic histiocytic panniculitis improved by combined CHOP and cyclosporin A treatment. In a 31-year-old Japanese man with cytophagic histiocytic panniculitis (CHP) remission was achieved by a combination of combined chemotherapy CHOP and cyclosporin A treatment. He was admitted to our hospital in January 1994 with recurrent high fever of 40.2 degrees C and tender and violaceous subcutaneous nodules on his trunk, arms and legs. He developed pancytopenia, hemorrhagic diathesis, liver dysfunction. Histological examination of the biopsied subcutaneous nodule revealed a lobular panniculitis with fat necrosis and a massive infiltration of histiocytes phagocytosing nuclear debris. He was treated initially with 40 mg/day prednisolone. However, following a reduction in prednisolone dosage, his symptoms reappeared. CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) therapy was then initiated. Three courses of CHOP treatment alleviated his symptoms and cyclosporin A was used to maintain his condition for 15 months. His medication was then discontinued and he has been in complete remission for 10 months. Combined treatment of cyclosporin A and CHOP combined chemotherapy was shown to be effective for this patient with severe CHP. ( info) |
2/134. Successful treatment of a patient with febrile, lobular panniculitis (Weber-Christian disease) with oral cyclosporin A: implications for pathogenesis and therapy. We report a 15-year-old Japanese girl with severe systemic Weber-Christian disease (WCD) who presented with acute onset of high fever associated with tender subcutaneous nodules. Laboratory tests showed an elevated serum concentration of lactate dehydrogenase (LDH), leukopenia, and coagulation abnormalities. The anti-nuclear and anti-dna antibodies were negative, and the serum pancreatic enzymes and alpha 1-antitrypsin levels were normal. pulse steroid therapy was not effective, and eventually cerebellar hemorrhage occurred. After initiation of oral cyclosporin A (CyA) therapy, fever came down and her clinical condition improved markedly. Extremely high serum concentrations of interferon-gamma (IFN-gamma) and soluble interleukin-2 receptor (sIL-2R) in this patient returned to normal with CyA therapy. These findings suggest that T-cell immune responses are involved in the pathogenesis of WCD, and that CyA is effective against the disease via suppression of T-cell reactions. ( info) |
3/134. Central serous retinopathy associated with Weber-Christian disease. PURPOSE: To report an association between central serous retinopathy and Weber-Christian disease. methods: A case report. RESULTS: Central serous retinopathy was noticed in a patient with Weber-Christian disease possibly secondary to vasculitis involving the choriocapillaris. CONCLUSIONS: Central serous retinopathy may be associated with an uncommon systemic condition like Weber-Christian disease. ( info) |
4/134. Suppurative granulomatous eosinophilic panniculitis: case report. A case of 12 year-old Nigerian male is presented. He had an unusual variant of Weber-Christian disease and manifested massive subcutaneous indurations and nodules limited to the cheeks, lips, left pectoral, infraclavicular and supraclavicular areas. A wedge biopsy revealed suppurative granulomatous eosinophilic panniculitis. Despite exhaustive investigations, no obvious trigger of the panniculitis could be identified. Response to corticosteroids and to empirical trials with other drugs was poor, and the outcome was fatal. We believe this is the first report from nigeria of this rare variant of Weber-Christian panniculitis in the paediatric age, and draw attention to the life-threatening nature of this disorder. ( info) |
5/134. Weber-Christian disease presenting with proptosis: a case report. Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid. ( info) |
6/134. Recurrent panniculitis in a man with asthma receiving treatment with leukotriene-modifying agents. Leukotriene-modifying drugs are novel agents introduced recently to treat asthma. Both 5-lipoxygenase inhibitors, such as zileuton, and leukotriene receptor antagonists, such as zafirlukast and montelukast, have proved effective in the treatment of asthma. To our knowledge, there have been no detailed reports regarding dermatologic manifestations of this class of drugs. This article describes an unusual case of erythema nodosum in a 46-year-old asthmatic man who received 2 different leukotriene modifiers. ( info) |
7/134. Systemic Weber-Christian disease complicated by partial transverse myelopathy. Weber-Christian disease is an inflammatory disorder of fatty tissue which usually presents with raised red tender nodules in the skin. Although there may be additional systemic upset, there are very few reports of neurological features associated with this condition. We report a patient with biopsy-confirmed systemic Weber-Christian disease in whom a transient partial myelopathy, of probable inflammatory origin, was the most prominent feature. Based on recent reports of the effects of immune mediators on neuronal function, a possible pathogenetic explanation for this syndrome is suggested. ( info) |
8/134. A case of cytophagic histiocytic panniculitis associated with exertional rhabdomyolysis. An 18-year-old man who suffered from panniculitis involving the entire left lower limb after exertional rhabdomyolysis is reported. A high fever (>39 degrees C) and leukocytosis (>20,000/microL) persisted for 1 week, and his general status deteriorated rapidly into pre-disseminated intravascular coagulation, complicated by pleural effusion and prolonged clotting time. His condition was dramatically improved by steroid pulse therapy and he has remained in good health for the 20 months since discharge. Histologic examination of subcutaneous tissue from the swollen left lower limb revealed pleomorphic small, medium or large lymphocytes, macrophages and neutrophils infiltrating the edematous subcutaneous adipose tissue in a lobular panniculitis-like pattern. The majority of inflammatory cells were T lymphocytes, with equal proportions of CD4 and CD8 cells. As polymerase chain reaction did not show bands suggesting T cell receptor gamma gene rearrangement, the proliferation of T lymphocytes was considered to be polyclonal. The T lymphocytes also expressed Fas ligand, suggesting the involvement of Fas-mediated cytotoxicity. This case may represent a new category of cytophagic histiocytic panniculitis induced by exertional rhabdomyolysis. ( info) |
9/134. Systemic Weber-Christian disease. BACKGROUND: Weber-Christian disease is a controversial entity that histologically presents as a lobular panniculitis. A systemic variant of this disorder, in which visceral fat is affected, has been reported. OBJECTIVE: A 29-year-old woman with Weber-Christian disease developed a rapidly enlarging abdominal mass during hospitalization for a flare of her skin condition. The mass resolved spontaneously. Radiographic studies demonstrated a mesenteric cyst, which was thought to represent involvement of mesenteric fat by Weber-Christian disease. CONCLUSION: The appearance of an abdominal or pelvic mass in a patient with Weber-Christian disease may be due to visceral involvement by the inflammatory process. ( info) |
10/134. 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. ( info) |