DRESS syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Yazan Daaboul, M.D.

Overview

DRESS syndrome is characterized by a prolonged latency period (2-8 weeks following the administration of triggering drug). If left untreated, DRESS syndrome self-resolves following the discontinuation of the triggering drug in the majority of cases, but clinical manifestations may persist up to 3 months and the long-term sequelae have not yet been identified. Complications of DRESS syndrome include visceral organ involvement and long-term autoimmune diseases. The prognosis of DRESS syndrome is generally good, and the case-fatality rate is approximately 10%. Factors associated with worse prognosis have not yet been established.

Natural History

  • DRESS syndrome is characterized by a prolonged latency period.
  • Clinical manifestations of DRESS syndrome are usually delayed. Earliest manifestations may appear 2-8 weeks following the administration of triggering drug.
  • Initially, patients usually develop non-specific signs and symptoms, namely fever and rash that involves the face, upper trunk, and upper extremities, making the early diagnosis of DRESS syndrome difficult upon patient presentation.
  • Additional clinical manifestations follow, and patients may subsequently develop lymphadenopathy, visceral disease (typically liver involvement), and worsening of the skin eruption.
  • The nature of the visceral involvement is thought to be associated with the identity of the triggering drug:[1]
  • Hepatic and GI involvement has been associated with abacavir
  • Renal involvement has been associated with allopurinol
  • Pulmonary involvement has been associated with abacavir and minocycline
  • If left untreated, DRESS syndrome self-resolves following the discontinuation of triggering drug in the majority of cases, but clinical manifestations may persist up to 3 months and the long-term sequelae have not yet been identified.

Complications

  • Organ involvement is considered the most important complication of DRESS syndrome.
  • Complications of DRESS syndrome include the following:
  • Long term complications often include the development of autoimmune diseases, such as:

Prognosis

  • The prognosis of DRESS syndrome is generally good.
  • The case-fatality rate of DRESS syndrome is approximately 10%. Mortality is most commonly due to fulminant hepatic failure.
  • Factors associated with worse prognosis of DRESS syndrome have not yet been established. Early drug discontinuation has been suggested as a favorable prognostic factor[2], whereas extent of affected body surface area is thought to be a poor prognostic factor.
  • Administration of corticosteroid therapy has not been demonstrated to be associated with reduced risk of death, [3], but patients who do not undergo slow corticosteroid tapering (over several weeks) are thought to be at an increased risk of relapse.

References

  1. Choudhary S, McLeod M, Torchia D, Romanelli P (2013). "Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome". J Clin Aesthet Dermatol. 6 (6): 31–7. PMC 3718748. PMID 23882307.
  2. Santiago F, Gonçalo M, Vieira R, Coelho S, Figueiredo A (2010). "Epicutaneous patch testing in drug hypersensitivity syndrome (DRESS)". Contact Dermatitis. 62 (1): 47–53. doi:10.1111/j.1600-0536.2009.01659.x. PMID 20136879.
  3. Chen YC, Chiu HC, Chu CY (2010). "Drug reaction with eosinophilia and systemic symptoms: a retrospective study of 60 cases". Arch Dermatol. 146 (12): 1373–9. doi:10.1001/archdermatol.2010.198. PMID 20713773.

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