Cyclophosphamide therapy for corticoresistant drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome in a patient with severe kidney and eye involvement and Epstein-Barr virus reactivation

Am J Kidney Dis. 2010 Mar;55(3):e11-4. doi: 10.1053/j.ajkd.2009.10.054. Epub 2010 Jan 27.

Abstract

DRESS (drug reaction with eosinophilia and systemic symptoms) syndrome is a severe adverse drug reaction with significant mortality, characterized by erythroderma, fever, lymphadenopathy, and visceral involvement. We report a case of multivisceral DRESS syndrome with posterior multifocal placoid pigment epitheliopathy and acute tubulointerstitial nephritis responsible for dialysis-dependent acute kidney failure in the context of reactivation of Epstein-Barr virus infection. Because of resistance of the skin and kidney manifestations to prolonged corticosteroid therapy, a 6-month course of oral cyclophosphamide resulted in complete recovery of all symptoms. To our knowledge, this is the first case showing the efficacy of cyclophosphamide in severe DRESS syndrome.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acute Kidney Injury / chemically induced*
  • Acute Kidney Injury / drug therapy*
  • Adrenal Cortex Hormones / therapeutic use
  • Cyclophosphamide / therapeutic use*
  • Drug Resistance
  • Drug-Related Side Effects and Adverse Reactions / drug therapy*
  • Drug-Related Side Effects and Adverse Reactions / etiology
  • Eosinophilia / chemically induced
  • Eosinophilia / drug therapy*
  • Epstein-Barr Virus Infections / chemically induced*
  • Eye Diseases / chemically induced
  • Eye Diseases / drug therapy*
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Middle Aged
  • Severity of Illness Index

Substances

  • Adrenal Cortex Hormones
  • Immunosuppressive Agents
  • Cyclophosphamide