A Case of RPGN associated with toxic epidermal necrolysis / 대한내과학회지

ABSTRACT

RPGN is a catastrophic form of acute glomerulonephritis characterized by an abrupt onset and rapid deterioration of renal function resulting in oliguria within weeks or months. RPGN 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 poststreptococcal glomerulonephritis, membranoproliferative glomerulonephritis, and IgA nephropathy, Goodpasture'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 erythrocytes 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 case documenting the presence of RPGN in patients with TEN. And there maybe some relations between PRGN and TEN which require further study.