Benign recurrent intrahepatic cholestasis (Summerskill and Walshe syndrome). Case report and revision of bibliography

ABSTRACT

We describe the case of a 58-year old woman who had four episodes of choluric jaundice and severe pruritus, starting at age 48. Each episode lasted about four months and had a spontaneous remission. During icteric episodes, conjugated bilirubinemia, alkaline phosphatase, and to a lower degree transaminases, were increased; white cell count was higher and there was a marked rise in sedimentation rate. Between such episodes there was a complete clinical remission, and laboratory alterations returned to normal, although sometimes a high alkaline phosphatase persisted. There was no history of previous drug intake known to produce these disorders. During the last icteric episode, an exploratory laparatomy, with roentgenographic study was done, which demonstrated that the extrahepatic biliary tract was intact. Histology revealed that the liver showed a preserved, regular architecture, of which the salient feature was intrahepatic cholestasis. Corticoids and cholestyramine were of little benefit for pruritus.