DRESS syndrome natural history, complications and prognosis: Difference between revisions
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{{DRESS syndrome}} | {{DRESS syndrome}} | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}} | ||
==Overview== | ==Overview== | ||
Revision as of 13:21, 20 July 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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
- ↑ 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.
- ↑ 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.
- ↑ 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.