Review
The DRESS Syndrome: A Literature Review

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Abstract

The Drug Reaction with Eosinophilia and Systemic Symptom (DRESS) is a severe adverse drug-induced reaction. Diagnosing DRESS is challenging due to the diversity of cutaneous eruption and organs involved. We used the RegiSCAR scoring system that grades DRESS cases as “no,” “possible,” “probable,” or “definite” to classify cases reported in the literature. We also analyzed the clinical course and treatments of the cases. A total of 44 drugs were associated with the 172 cases reported between January 1997 and May 2009 in PubMed and MEDLINE. The most frequently reported drug was carbamazepine, and the vast majority of cases were classified as “probable/definite” DRESS cases. Hypereosinophilia, liver involvement, fever, and lymphadenopathy were significantly associated with “probable/definite” DRESS cases, whereas skin rash was described in almost all of the cases, including “possible cases.” Culprit drug withdrawal and corticosteroids constituted the mainstay of DRESS treatment. The outcome was death in 9 cases. However, no predictive factors for serious cases were found. This better knowledge of DRESS may contribute to improve the diagnosis and management of this syndrome in clinical practice.

Section snippets

Materials and Methods

A systematic review of DRESS cases reported in the literature was carried out by searching PubMed-MEDLINE between January 1997 and May 2009. Search terms were “DRESS syndrome,” “drug reaction with eosinophilia and systemic symptoms,” “drug rash with eosinophilia and systemic symptoms,” “drug hypersensitivity and eosinophilia,” and “drug-induced hypersensitivity syndrome.” Publications were limited to the English and French languages.

We used the RegiSCAR's scoring system recently published to

Results

From a total of 131 independent published reports, 172 cases of DRESS were analyzed. Publications were excluded from analysis when they displayed data group summaries in which data could not be fully analyzed because the results were not assigned to a specific patient (Figure).

The drugs associated with DRESS and the distribution of the cases after RegiSCAR's score assignment are displayed in Table 2.12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35,

Discussion

In this review, we have done an extended analysis of 172 cases reported as DRESS or drug hypersensitivity syndrome in the literature by using the RegiSCAR scoring system.11

This review shows that DRESS is challenging to diagnose. Patients defined by the scoring system as “no/possible” cases of DRESS accounted for approximately one quarter of all the DRESS cases reported in the literature. However, cases classified as “probable/definite” by the RegiSCAR scoring system represented the vast

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    Funding: This work was funded by Institut de Recherche Internationale Servier.

    Conflict of Interest: Pr. Jean Claude Roujeau has provided punctual advice on cases of adverse reactions to drugs from more than 15 different pharmaceutical companies in the past 20 years. He has served as an expert on 3 trials related to severe drug reactions in US Courts. He is a member of advisory boards on Severe Cutaneous Adverse Reactions for Pfizer (2002-2007), Vertex (2008-ongoing), Servier (2008-ongoing), Boehringer Ingelheim (2010-ongoing), Roche (2010-ongoing). He has participated in the RegiSCAR research group, funded in part by several pharmaceutical companies (detailed list provided in publications by the RegiSCAR group, eg, Sassolas B et al. Clin Pharmacol Ther. 2010;88[1]:60-8). No other authors have any conflicts of interest to report.

    Authorship: All authors had access to the data and played a role in writing this manuscript.

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