RAMB - Maio / Junho 2016

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228

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ras

2016; 62(3):227-230

nopathy and lesions in target organs (liver, kidneys, lungs,

brain, muscles, thyroid, pancreas, heart), DRESS syn-

drome is a rare example of life threatening drug hyper-

sensitivity reaction.

1-3

The incidence of this syndrome is unknown. The fre-

quency varies depending on the drug used and the im-

mune status of the individual.

The period from drug exposure to onset of disease

can last 2 to 6 weeks, and may persist with a series of re-

missions and relapses. After discontinuation of the drug,

the average recovery time is 6-9 weeks. DRESS syndrome

has an approximate mortality rate of 10%.

2-4

Predictors

of poor prognosis have not been identified so far, but pa-

tients with increased liver impairment and advanced age

are at increased risk of death.

2,5

There are reports of cas-

es of autoimmune disease months to years after the res-

olution of the syndrome.

Given that this is a rare but severe disease, we report

a case of DRESS syndrome in a 20-year old female pa-

tient, black, former alcoholic and former user of cocaine,

with epilepsy and non-specified neuropsychiatric disor-

der, admitted to Hospital Municipal Odilon Behrens

(HMOB), Belo Horizonte, state of Minas Gerais, present-

ing fever of 38ºC, prostration and drowsiness. After two

days of onset of symptoms, the patient developed jaun-

dice (+/4), bipalpebral and lower limbs edema (++/4), mac-

ular erythema and lamellar desquamation in the face,

trunk, upper and lower extremities (Figures 1 and 2) and

bilateral cervical lymph node enlargement, with approx-

imately 1 cm in diameter, painless and mobile. The pa-

tient had been undergoing irregular home treatment

with diazepam, haloperidol and valproic acid for 4 weeks.

Initially, the symptoms were treated as soft tissue infec-

tion with prescription clavulanate amoxicillin and

azithromycin, suspended on hospital admission.

Laboratory tests performed on admission showed

normocytic and normochromic anemia, thrombocyto-

penia, leukocytosis without a left shift and with mild

neutropenia, atypical lymphocytosis and eosinophilia

(9%; 973/mm

), as well as elevated levels of C-reactive pro-

tein (CRP), transaminases (2 times the reference value),

alkaline phosphatase (ALP), gamma-glutamyl transpep-

tidase (GGT), bilirubin (direct and indirect) and chang-

es in kidney function. Serology for human immunode-

ficiency virus and hepatitis B and C were negative. Chest

radiography revealed no abnormalities, while abdomi-

nal ultrasound showed mild hepatomegaly and contract-

ed gallbladder.

In view of a possible diagnosis of DRESS syndrome,

the patient was hospitalized and corticosteroid therapy

with prednisone (40 mg once daily) was initiated. The drugs

that the patient used at home (valproic acid and haloper-

idol) were suspended, and only diazepam was continued.

After ten days of the introduction of corticosteroid

therapy, the patient progressed asymptomatic, afebrile,

with progressive improvement of skin lesions, even though

a scaly appearance continued. The dosage of liver trans-

aminases, ALP, GGT, bilirubin, CRP, lymphocytes and

eosinophils remained high, although lower than those at

admission. There was improvement in kidney function.

FIGURE 1

Diffuse desquamative erythema and face edema.

FIGURE 2

Diffuse erythema and edema in the lower limbs.