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DRESS syndrome

(Drug rash with Eosinophilia and Systemic Symptoms)

Dr Sampurna Roy MD                    

 

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DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms), is also referred to as drug-induced hypersensitivity syndrome.

DRESS syndrome is acronym for:

D =Drug

R =Reaction with

E= Eosinophilia and

S= Systemic

S= Symptoms

DRESS syndrome was initially recognized as a serious form of cutaneous drug adverse reaction.

Now it is regarded as a drug-related syndrome that can cause life-threatening organ dysfunctions.

Clinically, the skin lesion may present as urticarial and papular eruption,  erythema-multiforme-like pattern, erythroderma or erythematous and macular reaction pattern.

The cutaneous lesions usually involve the face, trunk, and proximal limbs.

The patients present with high fever, enlargement of the liver, spleen, and several peripheral lymph nodes.

       

Enlargement of the liver, spleen, and several peripheral lymph nodes.

Blood tests show marked eosinophilia, lymphocytosis, hyperbasophilic cells, and severe inflammation.

Histopathological features include dyskeratosis, interface changes, superficial perivascular dermatitis, focal spongiosis, lichenoid infiltrate, eosinophilic infiltration in some cases and rare presence of necrotic keratinocytes.

Dermal eosinophils are not prominent in all cases.

These features can be interpreted as a drug induced dermatitis.

 A)  

B)

All features of interface dermatitis are present in figures A) ; B) and C). In the image D) there is perivascular inflammatory infiltrate with sprinkling of eosinophils.

C)    

D)

Note: High eosinophil count is present in the peripheral blood smear of all patients in DRESS syndrome, but in histopathological examination of skin biopsy dermal eosinophils are not a prominent feature. 

 

The worldwide rate of mortality is about 10%. Drugs that usually cause DRESS are anticonvulsive drugs (carbamazepine, phenobarbital), and more recently new retroviral therapies.

The pathogenesis of DRESS is not yet fully understood, but it usually involve a combination of immune reactions, ethnic predisposition, genetically determined enzyme deficiencies and reactivation of herpes viruses (HHV-6, HHV-7, EBV, CMV).

 

Further reading:

Drug reaction with Eosinophilia and Systemic Symptoms (DRESS) / Drug-induced Hypersensitivity Syndrome (DIHS): a review of current concepts.

Drug reaction with eosinophilia and systemic symptoms (DRESS) .

Histopathologic spectrum of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS): a diagnosis that needs clinico-pathological correlation.

DRESS syndrome.

DRESS syndrome in a patient on sulfasalazine for rheumatoid arthritis.

Carbamazepine-induced drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome: report of four cases and brief review.

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS): a review.

Vancomycin-associated drug reaction with eosinophilia and systemic symptoms syndrome.

Prevalence of DRESS syndrome.

 

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Dr  Sampurna Roy  MD

Consultant Histopathologist (Kolkata - India)

 

 

 


 

 

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