OBJECTIVES & QUESTIONS
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
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?
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:
- Chinese herbal medicines
* Most commonly associated drugs
- 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?
- Enlarged lymph nodes
- Non-specific rash covering >50% body
- Papules, plaques, purpura
- Facial oedema
- Atypical lymphocytosis
- 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%)
What is the differential diagnosis of DRESS syndrome?
- Stevens Johnson Syndrome (SJS) / Toxic Epidermal Necrolysis (TEN)
- Acute generalized erythematous pustulosis
- Staphylococcal scalded skin syndrome
- Erythroderma (eczema, psoriasis)
- Angioimmunoblastic lymphadenopathy
- Idiopathic hypereosinophilic syndrome
- Polyarteritis nodosa
- Wegener granulomatosis
- Churg Strauss syndrome
- Systemic lupus erythematosus
- Kawasaki disease
- Primary HIV
- Human herpesvirus-6 (HHV-6)
- Epstein-Barr virus (EBV)
- Cytomegalovirus (CMV)
- Hepatitis A virus
- Hepatitis B virus