Drug related rash with eosinophilia and systemic symptoms (DRESS)

ICD-11

EH65

Synonyms

DRESS syndrome; drug-induced hypersensitivity syndrome; Drug induced delayed multi-organ hypersensitivity syndrome.

Epidemiology

Incidence 10/million/year
Male/female ratio 0.8; median age at diagnosis 53 years

Definition

Drug induced rash with blood eosinophilia and involvement of internal organs.

Aetiology & Pathogenesis

Examples for medications that may cause a DRESS are:

Antiarrhythmics: Mexiletin

Anticonvulsants: Carbamazepine, Phenytoin, Lamotrigine, Phenobarbital, Zonisamide

Anti-retrovirals: Abacavir, Nevirapine

Antibacterials: Amoxicillin, Levofloxacin, Minocycline

Other: Dapsone, Sulfonamides, Allopurinol, Vemurafenib, Cobimetinib

A possible association with reactivation of HHV-6 infections as well as genetic factors possible.

Laboratory & other workups

Eosinophilia (>1500/µl).

Leukocytosis (> 11x109/L).

Atypical lymphocytes circulating.

Depending on the affected organ increase of transaminases, creatinine kinase, creatinine, amylase and lipase.

Dermatopathology

The histological picture can be very diverse, including spongiosis, apoptotic keratinocytes, a superficial perivascular lymphocytic infiltrate and few dermal eosinophils.

Course

Depends on elimination of eliciting drug.

Clinical presentation

Acute onset of symptoms for up to six weeks after taking the drug.

Reduced general condition.

Generalized maculopapular rash, in the course of disease possible development of an exfoliative dermatitis or erythroderma.

Eventually mucosal enanthema.

Facial edema.

Fever > 38.5°C

Lymphadenopathy.

Involvement of minimum one internal organ (liver > kidney > lung, heart, pancreas).

Complications

Permanent damage of involved organs. Mortality up to 10%.

Diagnosis

Clinical picture, blood tests, histology.

Differential diagnosis

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN)

Acute generalized exanthematous pustulosis (AGEP)

Hypereosinophilic syndromes

Acute cutaneous lupus erythematosus

Prevention & Therapy

Drug withdrawal. Systemic glucocorticoids for several weeks

1.1.3.3 Drug related rash with eosinophilia and systemic symptoms (DRESS)