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78
Journal of The Association of Physicians of India ■ Vol. 63 ■ May 2015
Case Report
DRESS Syndrome: Drug Rash with Eosinophilia
and Systemic Symptoms
Hemant Thacker1, Manish Shah2, Rahul Tulle3
Abstract
DRESS Syndrome is caused by exposure to certain medications that may
cause fever, rash, inflammation of internal organs, lymphadenopathy
and characteristic hematologic abnormalities such as eosinophilia,
thrombocytopenia and atypical lymphocytosis. We herein report a case
of DRESS syndrome who presented with fever and rash, secondary to
sulfasalazine ingestion. Diagnosis of DRESS is often delayed, as several
diseases have clinical and laboratory features similar to DRESS syndrome.
Case History
A
51 year old female came to our
institution with complaints
of high grade fever since 3 days,
dry cough and prostration. Patient
had a history of rheumatoid
arthritis for which she was advised
Sulfasalazine 500 mg bid for the
last one month. On admission, the
patient looked sick, flushed and
febrile. Systemic examination was
not contributory.
Laboratory studies revealed
a leukocyte count of 10,330/
cumm with 67% neutrophils and
17% eosinophils, hemoglobin of
11.3 gm% and platelet count of
1 2 9 , 0 0 0 / c u m m . L i ve r e n z y m e s
we r e e l e va t e d w i t h a S G P T o f
157 U/L and SGOT of 124 U/L.
Te s t s f o r m a l a r i a a n d d e n g u e
were negative; Blood culture sent
on admission was subsequently
negative. Ultrasound abdomen
revealed a mild hepatomegaly.
Two days following admission,
the patient developed a red
macular rash with pruritus. The
rash initially appeared on the face
and trunk and within the next 36
hours had spread in a descending
fashion to all over the body. The
rash finally evolved to appear
as erythematous maculopapular
with edematous confluent lesions
involving entire body with more
severity over the face and limbs
(Figure 1).
Despite starting the patient
on ceftriaxone, broad spectrum
antibiotic, the fever was persistent.
A dermatologic consultation
was advised. The constellation
of erythematous edematous
rash on face, trunk and distal
extremities with eosinophilia
and atypical lymphocytosis and
raised transaminases; temporal
relation of rash occurring 4 weeks
after commencement of oral
sulfasalazine, clinched the diagnosis
in favour of DRESS, also called
drug-induced hypersensitivity
syndrome (DIHS). A skin biopsy
was advised for histopathological
conformation of DRESS syndrome,
but this was refused by the patient.
S u l f a s a l a z i n e wa s p r o m p t l y
discontinued and the patient was
started on oral prednisolone (1
mg/kg). The patient responded
dramatically with defervescence
f r o m t h e ve r y n e x t d a y a n d a
reduction in the rash. The liver
enzymes also began declining
t o n o r m a l i t y . T h e p a t i e n t wa s
discharged with gradual tapering
of steroid over a period of 6 weeks.
Discussion
Drugs incriminated commonly
for DRESS syndrome include
aromatic anticonvulsants
(phenobarbital, carbamazepine,
phenytoin, lamotrigine),
sulfonamides, allopurinol, antiretroviral drugs etc. It has been
suggested that concomitant Human
Herpes Virus-6 (HHV-6) infection
increases the risk to develop DRESS
syndrome.
The exact pathophysiologic
mechanism of DRESS syndrome
is not fully understood.
Postulated mechanisms include
defective detoxification of drug-
Fig. 1: Erythematous
maculopapular with
edematous confluent lesion
over upper limb
Consultant Physician and Cardio Metabolic Specialist, 2Consultant Dermatologist, 3DNB Medicine Resident,
Bhatia Hospital, Mumbai, Maharashtra
Received: 05.07.2013; Revised: 16.01.2014; Accepted: 17.01.2014
1
Journal of The Association of Physicians of India ■ Vol. 63 ■ May 2015
reactive metabolites resulting in
modification of cellular proteins,
targeting an autoimmune response.
There are 7 J-SCAR criteria for
diagnosis of DRESS syndrome: 2 (1)
maculopapular rash developing 3
weeks after beginning treatment
with the causative drug; (2)
prolonged clinical symptoms
after discontinuing the causative
drug; (3) fever >38°C (4) hepatic
abnormalities (5) leukocyte
abnormalities: leukocytosis,
eosinophilia (6) lymphadenopathy
( 7 ) H H V - 6 r e a c t i va t i o n . I f a l l
7 points are present then it is
termed as typical DIHS, if only
first 5 are present, atypical DIHS
is diagnosed. 79
Once the diagnosis of DRESS
syndrome has been established,
the next step in management
is immediate cessation of the
medication(s). Immediate
withdrawal of the causative
drug and initiation of systemic
corticosteroids is the mainstay
in the management of DRESS
syndrome. Thyroiditis has been
reported to develop in a subset of
patients as long-term sequelae.
drug reaction with a 10% mortality
rate. Clinicians must be aware of
this potentially fatal reaction and
its common culprit medications.
Prompt diagnosis using clinical
criteria, laboratory values, and
histopathology is imperative. The
aim of this article is to review
the available data regarding the
diagnosis and the management
of this probably underrecognised
subset of drug reaction.
Conclusion
References
DRESS syndrome is a drug
hypersensitivity reaction
with cutaneous and systemic
manifestations. DRESS syndrome
is a potentially fatal cutaneous
1.
Chauhan A, Anand S, Thomas S, et al.
Carbamazepine induced DRESS Syndrome;
J Assoc Physicians India 2010; 58.
2.
J o u r n a l o f Am e r i c a n Ac a d e my o f
Dermatology; 2013.