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DRESS syndrome

DRESS syndrome (Drug Rash with Eosinophilia and Systemic Symptoms) is an adverse reaction term that is currently used to describe a hypersensitivity reaction with an estimated mortality of up to 10%

Prevalence

10 / 100 000

N/A

US Estimated

N/A

Europe Estimated

Age of Onset

All ages

ICD-10

D72.12

Inheritance Pattern

Autosomal dominant

Autosomal recessive

Mitochondrial/Multigenic

X-linked dominant

X-linked recessive

Rare View

DRESS syndrome (Drug Rash with Eosinophilia and Systemic Symptoms) is an adverse reaction term that is currently used to describe a hypersensitivity reaction with an estimated mortality of up to 10%.

5 Facts you should know

FACT

1

A rare reaction to certain medications which includes characteristic blood abnormalities such as an abnormally high level of eosinophils, low number of platelets, and increased number of lymphocytes

FACT

2

The symptoms of DRESS syndrome usually begin 2 to 6 weeks but uncommonly up to 8 to 16 weeks after exposure to an offending drug

FACT

3

It primarily involves a widespread skin rash, fever, swollen lymph nodes, and characteristic blood abnormalities

FACT

4

DRESS is often complicated by potentially life-threatening inflammation of internal organs

FACT

5

The syndrome has about a 10% mortality rate

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DRESS syndrome is also known as...

DRESS syndrome is also known as:

  • drug-induced hypersensitivity syndrome

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While over 50 drugs have been identified to cause the DRESS syndrome, the most frequently reported drug classes associated with it are?

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Common signs & symptoms

Cutaneous

  • Widespread morbilliform or exfoliative rash
  • Facial edema (characteristic)
  • Pruritus

Systemic

  • Fever
  • Lymphadenopathy
  • Eosinophilia
  • Atypical lymphocytosis

Organ involvement

  • Liver (most common; hepatitis)
  • Kidneys (interstitial nephritis)
  • Lungs (pneumonitis)
  • Heart (myocarditis)
  • Thyroid (delayed autoimmune dysfunction) 

Current treatments

Immediate management

  • Prompt discontinuation of the offending drug

Medical Therapy

  • Systemic corticosteroids (moderate to severe cases)
  • Supportive care and organ-specific management

Severe or refractory cases

  • IV immunoglobulin (IVIG)
  • Other immunosuppressive agents (case-dependent)

References:

Kardaun SH, Sekula P, Valeyrie-Allanore L, et al. Drug reaction with eosinophilia and systemic symptoms (DRESS): an original multisystem adverse drug reaction. Results from the RegiSCAR study. <i>Br J Dermatol.</i> 2013;169(5):1071–1080. doi:10.1111/bjd.12501 Bocquet H, Bagot M, Roujeau JC. Drug-induced pseudolymphoma and drug hypersensitivity syndrome (Drug Rash with Eosinophilia and Systemic Symptoms: DRESS). <i>Semin Cutan Med Surg.</i> 1996;15(4):250–257. doi:10.1016/S1085-5629(96)80038-1 Shiohara T, Kano Y. Drug reaction with eosinophilia and systemic symptoms (DRESS): incidence, pathogenesis and management. <i>Expert Opin Drug Saf.</i> 2017;16(2):139–147. doi:10.1080/14740338.2017.1272785 Chen YC, Cho YT, Chang CY, Chu CY. Drug reaction with eosinophilia and systemic symptoms: a comprehensive review. <i>J Clin Med.</i> 2016;5(11):123. doi:10.3390/jcm5110123 Cho YT, Yang CW, Chu CY. Drug reaction with eosinophilia and systemic symptoms (DRESS): an interplay among drugs, viruses, and immune system. <i>Int J Mol Sci.</i> 2017;18(6):1243. doi:10.3390/ijms18061243

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