[A triple association: renal malacoplakia, bilateral vulvar hypertrophy, upper limb algodystrophy].

The authors report a case of malakoplakia of the left renal parenchyma, an uncommon site for an inflammatory disease first described in the bladder by Michaelis and Guttmann in 1902 and Von Hansmann in 1903. This case was observed in a 24-year-old girl with no urological history, presenting with bilateral vulval hypertrophy and reflex sympathetic dystrophy of the upper limbs, in whom malakoplakia of the left kidney was discovered incidentally. Intravenous urography showed two large kidneys with a stretched appearance of the renal pelvis. Pathological examination of the partial nephrectomy specimen revealed the diagnosis of malakoplakia, an inflammatory diseases of the renal parenchyma. The pathogenesis has been only partially elucidated, but probably involves a disorder of macrophage function.