J Korean Pediatr Soc.
1978 Sep;21(9):600-606.
A case of Weber-Christian Disease with nephrotic syndrome
- Affiliations
-
- 1Department of pediatrics, College of Medicine, Seoul National University, Korea.
- 2Department of Pathology, College of Medicine, Seoul National University, Korea.
Abstract
- A case of weber-Christian disease with nephrotic syndrome is presented. A 6-year-old boy was refered for further evaluation of nephrotic syndrome and multiple nodules in all extremities and trunk in Nov. 1975. In Mar. 1974, he experienced generalized edema and proteinura. So, celestone (betamethasone) medication started under impression of nephrotic syndrome. Initially, the proteinuria disappeared in response to large dose of celestone, but recurred when the dose of it was reduced. Three more subsequent relapse of nephrotic syndrome developed during 9 months inspite of continuous steroid medication. In Mar. 1975, crops of subcutaneous nodules of varing sized appeared in the shoulder and limbs. Those nodules resolved progressively without medication, but small bean sized nodules remained. In Nov. 1975, he complained of pain in flank and both lower extremities. Multiple nodules were palpable in the trunk and iimbs. High fever also occured. A skin biopsy from the left forearm revealed nonsuppurative panniculitis, consistent with Weber-Christian disease, macrophage stage. A percutaneous renal biopsy revealed (Epi-) membranous glomerulonephritis. It is difficutlt to speculate an etiological relationship between panniculitis and renal disorder in the present case. He received prednisolone alternately further 15 months. Chemical remission occured after 2 months of prednisolone therapy. He has remained free of proteinuria for 9 months after discontinuance of prednisolone therapy. Subcutaneous nodules of panniculitis resolved progressively, but some of them remained in the abdominal wall.
