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Introduction & Definition

What is DRESS syndrome?

  • DRESS syndrome stands for drug reaction with eosinophilia and systemic symptoms syndrome
  • It is a life-threatening drug-induced hypersensitivity syndrome characterized by a cutaneous presentation and internal organ involvement
Epidemiology Clinical Course Prognosis

How common Is DRESS syndrome?

  • The true incidence is unknown
  • Best estimate in the overall population is between 1 in 1000 to 10,000 drug exposures

What is the prognosis of DRESS syndrome?

  • Most patients recover completely after drug withdrawal and appropriate therapy
  • Some patients suffer from chronic complications such as diabetes mellitus or renal impairment
  • The mortality rate has not been clearly determined but approaches 10% in small observational studies

What are the common causes of death in DRESS syndrome?

  • Common causes include:
    • Acute liver failure
    • Multiorgan failure
    • Fulminant myocarditis
    • Haemophagocytosis (HLH)


What are the causes of DRESS syndrome?

  • A wide number of medications have been reported to precipitate DRESS
  • Medications classically associated include:
  • Carbamazepine*
  • Lamotregine
  • Phenobarbitol
  • Phenytoin
  • Gabapentin
Antibacterials / anti-TB
  • Amoxicillin
  • Levofloxacin
  • Tazocin
  • Ethambutol
  • Isoniazid
  • Rifampicin
  • Nevirapine
  • Abacavir
  • Telaprevir
  • Paracetamol
  • Diclofenac
  • Ibuprofen
  • Dapsone
  • Omeprazole
  • Allopurinol*
  • Chinese herbal medicines

* Most commonly associated drugs

What is the pathophysiology of DRESS syndrome?
  • Precise pathogenesis remains elusive but thought to be multifactorial with both immunologic and nonimmunologic factors
  • Drugs or their metabolites may accumulate in some persons due to dysfunctional drug detoxification pathways;
  • May evoking drug-specific T lymphocytes in persons with certain genetic backgrounds
  • Viral reactivation is also thought to play significant role:
    • Result from a direct effect of the inciting drugs or their metabolites or from a “cytokine storm” caused by anti-drug immune responses
    • Viruses implicated include cytomegalovirus (CMV), Epstein-Barr virus (EBV) and human herpesvirus-6 (HHV 6)
    • May induce robust anti-viral responses contributing to the development of the disease

What is the usual course of DRESS syndrome?

  • Characteristic feature of the clinical course of DRESS syndrome is a delayed onset and a prolonged and protracted evolution of the disease:
    • Typically develop symptoms and signs of drug reaction at least 3 weeks after the start of the eliciting drug
    • Latency period longer than of SJS/TEN
  • May be preceded by prodromal illness
  • Duration of illness is usually more than 15 days with waxing and waning course

What are the clinical features of DRESS syndrome?

Inflammatory Features
  • Fever
  • Enlarged lymph nodes
Cutaneous Features
  • Non-specific rash covering >50% body
  • Papules, plaques, purpura
  • Facial oedema
Haematological Features
  • Eosinophilia
  • Atypical lymphocytosis
Organ Failure / Other
  • Hepatic dysfunction (hepatocellular or cholestatic)
  • Renal failure
  • ARDS and respiratory failure
  • Myocarditis and cardiac failure
  • Haemophagocytic Lymphohistiocytosis (HLH)

What are the characteristic features of the rash in DRESS syndrome?

  • Characteristics of the rash are diverse:
    • Morbilliform (measles-like) eruption (in 80%):
    • Fine erythematous macules and papules
    • Distributed over the trunk
    • Varying morphology including target lesions, blisters and pustules
  • Erythroderma or exfoliative dermatitis involving > 90% body surface area (in about 10%)
  • Facial swelling (in 30%)
  • Mucosal involvement – lips, mouth, throat, genitalia (in 25%)

Differential Diagnosis

What is the differential diagnosis of DRESS syndrome?

Dermatological Disorders
  • Stevens Johnson Syndrome (SJS) / Toxic Epidermal Necrolysis (TEN)
  • Acute generalized erythematous pustulosis
  • Staphylococcal scalded skin syndrome
  • Erythroderma (eczema, psoriasis)
Haematological Conditions
  • Angioimmunoblastic lymphadenopathy
  • Lymphoma
  • Idiopathic hypereosinophilic syndrome
  • Polyarteritis nodosa
  • Wegener granulomatosis
  • Churg Strauss syndrome
  • Systemic lupus erythematosus
  • Kawasaki disease
Acute viral Infection
  • Primary HIV
  • Human herpesvirus-6 (HHV-6)
  • Epstein-Barr virus (EBV)
  • Cytomegalovirus (CMV)
  • Hepatitis A virus
  • Hepatitis B virus
  • Influenza
Work-Up Summary
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Laboratory Investigations

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Imaging Investigations
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Management Summary
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Critical Care Admission & Referral
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Supportive Management
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Specific Management
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The Guidewire
Trainee in ICM & Anaesthesia


The Guidewire
Trainee in ICM & Anaesthesia