DRESS syndrome medical therapy

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

Overview

Drug withdrawal is the first step in the management of DRESS syndrome. Supportive care is considered the mainstay of therapy, and hospitalization and administration of pharmacologic agents is usually necessary. Medical therapy for the management of DRESS syndrome usually include topical and/or systemic corticosteroids, antipyretic agents, and symptomatic management of exfoliative dermatitis (fluids, nutritional support, management of electrolyte imbalances, and sepsis prevention). Additional therapies have been attempted with variable success, including the use of N-acetylcysteine, intravenous immunoglobulins, and cyclosporin.

Medical Therapy

  • Drug withdrawal is the first step in the management of DRESS syndrome.
  • There are no randomized controlled trials that evaluate the efficacy and safety of drugs in the management of DRESS syndrome. The current approach to management of DRESS syndrome is mostly based on case series and expert consensus.
  • Patients with severe skin eruptions are usually admitted to the hospital for close monitoring and fluid/electrolyte management.
  • Supportive care is considered the mainstay of therapy, and hospitalization and administration of pharmacologic agents is usually necessary. Data from randomized clinical trials regarding the efficacy of immunosuppressive therapy in the management of DRESS syndrome is not yet available.
  • Persistent DRESS syndrome often requires prolonged therapy (possible beyond 1 year).
  • Patients hospitalized for DRESS syndrome require multidisciplinary care, including consultation by a dermatologist, hepatologist, and liver transplant specialist.[1]
  • The following drugs have been used in the management of DRESS syndrome:

1. Symptomatic relief

  • Note: Avoid antipyretic drugs that are cleared by the liver (e.g. Acetaminophen) if liver injury is suspected and drugs cleared by the kidney (e.g. NSAIDs) if renal injury is suspected

2. Exfoliative dermatitis

  • Management of exfoliative dermatitis is similar to management of burns:[2]
  • Warming
  • Management of electrolyte derangements
  • Intake of high-caloric diet
  • Sepsis prevention

3. Liver involvement

  • Unknown benefit of systemic corticosteroids
  • Referral to liver transplant in cases of severe hepatic injury (> 3x ULN of LFTs)

4. Lung involvement

  • Systemic corticosteroids: Prednisone 0.5-2 mg/kg/day (or equivalent) IV until clinical improvement THEN tapered slowly over 2-3 months[3]

5. Renal involvement

  • Systemic corticosteroids: Prednisone 0.5-2 mg/kg/day (or equivalent) IV until clinical improvement THEN tapered slowly over 2-3 months[3]

Other Pharmacologic Agents

Other pharmacologic agents that have been used in the management of DRESS syndrome are the following:

References

  1. Davern TJ (2012). "Drug-induced liver disease". Clin Liver Dis. 16 (2): 231–45. doi:10.1016/j.cld.2012.03.002. PMID 22541696.
  2. Roujeau JC (1999). "Treatment of severe drug eruptions". J Dermatol. 26 (11): 718–22. PMID 10635613.
  3. 3.0 3.1 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.
  4. Zuliani E, Zwahlen H, Gilliet F, Marone C (2005). "Vancomycin-induced hypersensitivity reaction with acute renal failure: resolution following cyclosporine treatment". Clin Nephrol. 64 (2): 155–8. PMID 16114793.
  5. Joly P, Janela B, Tetart F, Rogez S, Picard D, D'Incan M; et al. (2012). "Poor benefit/risk balance of intravenous immunoglobulins in DRESS". Arch Dermatol. 148 (4): 543–4. doi:10.1001/archderm.148.4.dlt120002-c. PMID 22508885.
  6. Tas S, Simonart T, Heenen M (2001). "Angio-oedema caused by high doses of N-acetylcysteine in patients with anticonvulsant hypersensitivity syndrome". Br J Dermatol. 145 (5): 856–7. PMID 11736926.
  7. Tas S, Simonart T (2003). "Management of drug rash with eosinophilia and systemic symptoms (DRESS syndrome): an update". Dermatology. 206 (4): 353–6. doi:69956 Check |doi= value (help). PMID 12771485.