0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Research Letters |

Initial Presentation of DRESS: Often Misdiagnosed as Infections FREE

Haur Yueh Lee, MBBS, MRCP; Sarah Walsh, MB BCh, BaO, MRCP; Daniel Creamer, MD
[+] Author Affiliations

Author Affiliations: Department of Dermatology, King's College Hospital, Denmark Hill, London, England.


Arch Dermatol. 2012;148(9):1085-1087. doi:10.1001/archdermatol.2012.1079.
Text Size: A A A
Published online

Drug reaction eosinophilia and systemic symptoms (DRESS) is a severe cutaneous event characterized by a skin eruption, fever, hematologic abnormalities (eosinophilia or atypical lymphocytes), and internal organ involvement.1 DRESS may be complicated by multiple-organ failure requiring treatment in the intensive care unit.2 The overall mortality rate has been estimated at 10%,1,3 although more recent studies have suggested a rate nearer 2%.4 Early recognition of this syndrome, discontinuation of treatment with the suspected drug, and the institution of supportive and/or specific measures are vital.

However, the heterogeneity of initial presentation often results in the patient being initially managed by nondermatologists. In DRESS, the latency period is long (delay between initiation of treatment with the culprit drug and onset of reaction), between 3 weeks and 2 months, which further obscures drug culpability. The failure to recognize and manage DRESS as an adverse drug reaction was recently proposed in the Archives as a “Practice Gap.”5 The aim of our study was to review the presentation, initial diagnosis, and early management of DRESS in our institution and to determine if such a practice gap exists.

A retrospective review was performed on cases of DRESS managed in our institution from 2005 through 2011. All patients were recruited onto the SCAR-UK registry (Severe Cutaneous Adverse Reactions, United Kingdom). All cases were defined as probable or definite DRESS based on the RegiSCAR diagnostic criteria.6 Patients were either admitted following presentation or were already inpatients at the time of diagnosis. Case records were systematically reviewed, and data on initial clinical presentation, laboratory parameters, initial diagnosis, and treatment were collected. Diagnostic lag was defined as the interval from initial diagnosis at presentation to time of DRESS diagnosis. This study was approved by our institutional review board at King's College Hospital, London, England.

There were a total of 26 patients with DRESS included in the analysis (11 male; 15 female) with a mean age of 42 years (age range, 7-73 years). The validation scores for DRESS cases according to RegiSCAR criteria are listed shown in Table 1. The clinical features and laboratory findings at presentation are summarized in Table 1. Fever and malaise affected more than 75% of patients (Table 2). Seventeen patients initially presented to medical departments (internal medicine, 7; dermatology, 3; rheumatology, 2; hepatology, 2; pediatrics, 1; hematology, 1; and chest medicine, 1). Four patients presented to neurosurgery; 2 patients were on the intensive care ward; and 3 patients were initially seen in the emergency department.

Table Graphic Jump LocationTable 1. Validation of Cases According to the RegiSCAR6 DRESS Scoring Systema
Table Graphic Jump LocationTable 2. Initial Features on Presentation

The initial diagnosis was presumed to be solely infection in 13 of 26 patients (50%), which led to treatment with antibiotics (Figure). Lymphoma and drug hypersensitivity were the other initial diagnoses suspected (Figure). The mean lag between the initial diagnosis and the diagnosis of DRESS was 1.7 days. The diagnostic accuracy varied over the study period (2005-2011), as shown in the Figure. The proportion of cases presumed to be infection-related decreased from 0.6 in the 2005-2007 period to 0.5 in the 2007-2009 period to 0.38 in the 2010-2011 period.

Place holder to copy figure label and caption
Graphic Jump Location

Figure. Breakdown of initial diagnoses over the study period.

The clinical presentation of DRESS is heterogeneous. The constellation of fever, constitutional symptoms, eruption, and multiple-organ involvement led the initial consulting doctors in our study to consider an infectious illness as the primary diagnosis in 13 of 26 patients. Infection was also implicated in an additional 6 patients, although other differential diagnoses were considered. Drug hypersensitivity was deemed as the most likely diagnosis in only 7 patients (27%), and an initial diagnosis of DRESS was never made.

The initial misdiagnosis of DRESS led to an average diagnostic delay of 1.7 days. This short delay is likely attributed to the fact that in our institution, liaison inpatient dermatology services are available 24 hours a day. On analyzing the diagnostic accuracy over the study period, we found that an increasing proportion of cases were considered to have an underlying drug cause. This improvement most likely reflects our efforts to educate our medical colleagues about the drug-induced dermatoses and dissemination of information on DRESS in particular.

Our study further illustrates how difficult the diagnosis of DRESS can be. A close cooperation between dermatologists and other hospital physicians may decrease the delay in diagnosis as well as bring about a greater awareness of this syndrome.

Correspondence: Dr Creamer, Department of Dermatology, King's College Hospital, Denmark Hill, London, SE5 9RS United Kingdom (daniel.creamer@nhs.net).

Author Contributions: Drs Lee and Creamer had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Creamer. Acquisition of data: Lee, Walsh, and Creamer. Analysis and interpretation of data: Lee, Walsh, and Creamer. Drafting of the manuscript: Lee. Critical revision of the manuscript for important intellectual content: Walsh and Creamer. Administrative, technical, and material support: Walsh. Study supervision: Walsh and Creamer.

Financial Disclosure: None reported.

Bocquet H, Bagot M, Roujeau JC. Drug-induced pseudolymphoma and drug hypersensitivity syndrome (drug rash with eosinophilia and systemic symptoms: DRESS).  Semin Cutan Med Surg. 1996;15(4):250-257
PubMed   |  Link to Article
Eshki M, Allanore L, Musette P,  et al.  Twelve-year analysis of severe cases of drug reaction with eosinophilia and systemic symptoms: a cause of unpredictable multiorgan failure.  Arch Dermatol. 2009;145(1):67-72
PubMed   |  Link to Article
Chen YC, Chiu HC, Chu CY. Drug reaction with eosinophilia and systemic symptoms: a retrospective study of 60 cases.  Arch Dermatol. 2010;146(12):1373-1379
PubMed   |  Link to Article
Kardaun SH, Sekula P, Mockenhaupt M,  et al.  Drug reaction with eosinophilia and systemic symptoms (DRESS): results from the RegiSCAR.  Eur Ann Allergy Clin Immunol. 2010;42(1):45
England Owen C, Stratman EJ. Failure to recognize and manage patients with DRESS.  Arch Dermatol. 2010;146(12):1379
PubMed   |  Link to Article
Kardaun SH, Sidoroff A, Valeyrie-Allanore L,  et al.  Variability in the clinical pattern of cutaneous side-effects of drugs with systemic symptoms: does a DRESS syndrome really exist?  Br J Dermatol. 2007;156(3):609-611
PubMed   |  Link to Article

Figures

Place holder to copy figure label and caption
Graphic Jump Location

Figure. Breakdown of initial diagnoses over the study period.

Tables

Table Graphic Jump LocationTable 1. Validation of Cases According to the RegiSCAR6 DRESS Scoring Systema
Table Graphic Jump LocationTable 2. Initial Features on Presentation

References

Bocquet H, Bagot M, Roujeau JC. Drug-induced pseudolymphoma and drug hypersensitivity syndrome (drug rash with eosinophilia and systemic symptoms: DRESS).  Semin Cutan Med Surg. 1996;15(4):250-257
PubMed   |  Link to Article
Eshki M, Allanore L, Musette P,  et al.  Twelve-year analysis of severe cases of drug reaction with eosinophilia and systemic symptoms: a cause of unpredictable multiorgan failure.  Arch Dermatol. 2009;145(1):67-72
PubMed   |  Link to Article
Chen YC, Chiu HC, Chu CY. Drug reaction with eosinophilia and systemic symptoms: a retrospective study of 60 cases.  Arch Dermatol. 2010;146(12):1373-1379
PubMed   |  Link to Article
Kardaun SH, Sekula P, Mockenhaupt M,  et al.  Drug reaction with eosinophilia and systemic symptoms (DRESS): results from the RegiSCAR.  Eur Ann Allergy Clin Immunol. 2010;42(1):45
England Owen C, Stratman EJ. Failure to recognize and manage patients with DRESS.  Arch Dermatol. 2010;146(12):1379
PubMed   |  Link to Article
Kardaun SH, Sidoroff A, Valeyrie-Allanore L,  et al.  Variability in the clinical pattern of cutaneous side-effects of drugs with systemic symptoms: does a DRESS syndrome really exist?  Br J Dermatol. 2007;156(3):609-611
PubMed   |  Link to Article

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections
PubMed Articles
Epidemiology of cutaneous drug-induced reactions. G Ital Dermatol Venereol 2014;149(2):207-18.