독성 표피 괴사융해증과 동반된 급속 진행성 사구체신염 1 예 = A Case of RPGN associated with toxic epidermal necrolysis

다국어 초록 (Multilingual Abstract)

RPGN is a catastrophic form of acute glomerulone-phritis characterized by an abrupt onset and rapid deterioration of renal function resulting in o1iguria within weeks or months, BPGN is seen in a variety of systemic disorders, including systemic lupus erythematosus, poly arteritis nodosa, Wegener's granulomatosis and subacute bacterial endocarditis. In addition, RPGN is seen in association with a variety of primary renal diseases such as poststreptacoccal glomrulonephritis, merrkranoproli-ferative glomerulonephritis, and IgA nephropathy, Good-pasture's syndrome. Toxic epidermal necrolysis (TEN) is a drug induced life threatening disease characterized by extensive epidermal detachment, necrosis, and mucosal erosion. TEN may involve liver, lung, intestine, and kidney. But renal involvement has seldom been reported. We report on a 63-year-old patient who developed a RPGN with a TEN. Renal biopsy showed pauci-immune crescentric glomerulonephritis and skin biopsy showed edematous change with extravasated erytkvcytes in upper dermis and several individually necrotic keratinocytes. ANCA and FANA test was negative. Our patient recovered renal function with steroid pulse therapy. The pathophysiology of TEN is unresolved but abnormal cytokine release (e.g., tumor necrosis factor) has been implicated in pathogenesis of TEN. Because various cytokines have direct toxic effect on kidney structure, the tubular and glomerular damage may be related to the cytokines involved in TEN. To our knowledge, this is the first documenting the presence of RPGN in patients with TEN. And there maybe some relations between PRGN and TEN which require further study.