RAMB - Maio / Junho 2016

rug

reaction

with

eosinophilia

and

systemic

symptoms

(DRESS

syndrome

)

ssoc

ras

2016; 62(3):227-230

229

The patient was referred to the internal medicine outpa-

tient clinic at HMOB for assessment until cure of DRESS

syndrome.

onclusion

Drugs associated with DRESS syndrome include: phenyt-

oin, carbamazepine, phenobarbital, valproic acid, ami-

triptyline, fluoxetine, piroxicam, diclofenac, naproxen,

ibuprofen, captopril, atenolol, propiutiouracil, sulfon-

amides, allopurinol and some antiviral/antibiotic agents,

such as amoxicillin alone or in combination with clavu-

lanate, and azithromycin. In 10 to 20% of cases, the drug

possibly responsible for the syndrome may not be iden-

tified in the patient’s medical history.

1,5,7,8

All the drugs

used by our patient could have triggered the reaction. The

causality is smaller for haloperidol and diazepam, due to

lack of studies showing this association.

In the pathogenesis of the disease, a particular im-

mune response to the drug with expansion of T lympho-

cytes has been demonstrated. Studies indicate that the

reactivation of human herpes virus, cytomegalovirus and

Epstein-Barr virus may be related to the pathophysiolo-

gy. Alleles of the human leukocyte antigen (HLA) have

been associated with susceptibility to DRESS, suggesting

that genetic factors can also be involved.

2,5

DRESS should be suspected in a patient who receives

medical treatment and who presents with the following

signs and symptoms: skin rash, fever (38 to 40°C), facial

edema and lymphadenopathy. In 20 to 30% of patients,

erythema progresses to exfoliative dermatitis.

These find-

ings, including peeling after morbilliform skin rash, were

clinical manifestations presented by our patient.

The diagnostic criteria for DRESS syndrome are pre-

sented in Chart 1. There is no consensus on the criteria,

because they lack specific clinical and laboratory charac-

teristics to establish the diagnosis.

Patients with DRESS should be monitored for pro-

gression of symptoms and laboratory abnormalities sug-

gestive of visceral involvement. This patient progressed

with changes in liver and kidney function. There were no

other changes suggestive of organ damage.

Differential diagnoses include: Stevens Johnson/tox-

ic epidermal necrolysis, hypereosinophilic syndrome and

Kawasaki disease.

Drug etiologies are present in DRESS

syndrome, as mentioned above, and in Stevens Johnson/

toxic epidermal necrolysis, especially sulfonamides, anti-

convulsants, NSAIDs and Penicillin.

Hypereosinophilic

syndrome is triggered by an exaggerated inflammatory re-

sponse at the expense mainly of interleukin five (IL-5).

Cytokines, endothelins and vasoactive mediators trigger

impairment of vascular integrity in Kawasaki disease.

Mucocutaneous manifestations are present in all of

these diseases; hematologic abnormalities, such as eosin-

ophilia and presence of atypical lymphocytosis, may be

found in DRESS and hypereosinophilic syndrome. Lymph-

adenopathy, hepatitis, and damage to other organs can

be related to all the diseases described. Differences in clin-

ical and laboratory presentations among these differen-

tial diagnoses are summarized in Chart 2.

Treatment of DRESS primarily involves the removal

of possibly involved drugs, the use of topical (for itching

and inflammation of the skin) and especially systemic

corticosteroids, as well as supportive measures. Due to

the possible genetic correlation, family members should

also be advised to avoid using the causal drug.

The diagnosis of DRESS syndrome is a challenge

due to unspecific manifestations that may hinder its rec-

ognition. Thus, to address and discuss this topic is ex-

tremely important, considering the potential lethality

of a treatable syndrome. Recognizing the occurrence of

DRESS syndrome and starting treatment as soon as pos-

sible is crucial to reduce the risk of mortality and im-

prove prognosis.

CHART 1

Diagnostic criteria of DRESS.

1. Drug-related skin rash

2. Hematologic abnormalities:

Eosinophilia greater or equal to 1,500/mm

or atypical

lymphocytosis

3. Systemic involvement:

Lymphadenopathy greater or equal to 2 cm in diameter or

Hepatitis (increased transaminases with values twice higher than

normal) or

Interstitial nephritis or

Interstitial pneumonitis or

Carditis

Diagnosis is confirmed in the presence of three criteria.

esumo

Reação a drogas com eosinofilia e sintomas sistêmicos

(síndrome DRESS)

Objetivo:

fazer uma revisão da reação de hipersensibili-

dade a drogas denominada reação a drogas com eosino-

filia e sintomas sistêmicos (síndrome DRESS), com base

em um relato de caso clínico. Pretende-se ainda discutir

a dificuldade e importância de seu reconhecimento, uma