|Year : 1984 | Volume
| Issue : 1 | Page : 49-50
Weber-Christian disease (febrile relapsing non-suppurative nodular panniculitis) (a case report).
NK Singh, DS Singh
N K Singh
|How to cite this article:|
Singh N K, Singh D S. Weber-Christian disease (febrile relapsing non-suppurative nodular panniculitis) (a case report). J Postgrad Med 1984;30:49-50
|How to cite this URL:|
Singh N K, Singh D S. Weber-Christian disease (febrile relapsing non-suppurative nodular panniculitis) (a case report). J Postgrad Med [serial online] 1984 [cited 2022 Aug 18 ];30:49-50
Available from: https://www.jpgmonline.com/text.asp?1984/30/1/49/5496
Weber-Christian disease is a rare and poorly understood disorder of the adipose tissue. It may be the results of several known and possibly many more unknown causes. In 1925, Weber first described a case as "relapsing non-suppurative nodular panniculitis" and Christian, in 1928, added "febrile" to this designation. Subsequently, the eponym Weber-Christian disease was introduced. Rarity of this condition and good therapeutic response to corticosteroid prompted us to report this case and highlight the clinical and diagnostic features of this disease.
A 20 year old female was hospitalized with 12 days' history of high grade fever and multiple, painful nodules on the trunk and lower extremities. She also complained of pain in both the ankles and elbow joints for the same duration. On direct interrogation, she gave history of 2 episodes of similar illness in the past over a period of 2 years. Each episode lasted for about 1 month. She denied any history of abdominal pain.
Examination of the patient revealed a thin built lady having pulse rate of 120/minute, oral temperature of 1000F and moderate pallor. There were multiple, tender, firm nodules of 1-2 cm in diameter, spread over the trunk and lower extremities. There was no joint swelling. Systemic examination was unremarkable.
Laboratory investigations revealed total WBC count of 13,000/cmm with differential leucocyte count of P-70%, L-27% and E-3%; Haemoglobin was 5.5 gm% and ESR-30 mm/hour (Wintrobe method). Tests for rheumatoid factor and L.E. cells were negative. Serial estimations of serum and urinary amylase were normal. Paper electrophoresis of serum proteins revealed mild. elevation of alpha-2 globulin. Barium meal contrast study of the upper gastro-intestinal tract showed no evidence of pancreatic malignancy. Biopsy from the skin nodule showed inflammatory cells in the fat tissue together with foci of fat necrosis [Fig. 1].
The patient had good therapeutic response with corticosteroids and other supportive measures. Prednisolone was given in a dose of 40 mg/day in divided doses for 1 week and then gradually tapered off over a period of 3 weeks. Two years follow-up information of the present case revealed no relapse of the disease.
Weber-Christian disease is a rare disorder of subcutaneous fat tissue. The exact aetio-pathogenesis of this condition is largely unknown. Some of the aetiological factors incriminated are immunologically mediated vasculitis and steroid withdrawl. A number of cases have been reported with other disease processes e.g. systemic lupus erythematosus, pancreatitis and pancreatic carcinoma. In the case under report, no definite aetiological factor could be established.
Clinically, it is characterised by acute febrile illness associated with recurrent crops of multiple, tender, subcutaneous nodules or plaques occurring most commonly on the thighs and legs but may involve the arms and sometimes also the trunk and face. Usually over a period of few weeks the nodules subside. The disease tends to recur at an interval of weeks or months. Rarely, visceral involvement may also occur which may prove to be fatal.
Histopathological changes of subcutaneous nodules in acute stage of the disease comprise of focal necrosis of fat cells with infiltration by polymorphonuclear leucocytes and lymphocytes. Later on, macrophages invade the fat lobules. Following the inflammatory phase, there is replacement of adipose tissue by fibrosis. Rarely, there may be associated vasculitis.
Good therapeutic response with corticosteroids and ACTH have been reported by various workers., Present case also had favourable therapeutic response to corticosteroid and had no recurrence of the disease in a 2 year follow-up period.
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