Drug reaction with eosinophilia and systemic symptoms (DRESS): an original multisystem adverse drug reaction. Results from the prospective RegiSCAR study
TL;DR: Cases of severe drug hypersensitivity, demonstrating a variable spectrum of cutaneous and systemic involvement, are reported under various names, especially drug reaction with eosinophilia and systemic symptoms (DRESS).
Abstract: BackgroundCases of severe drug hypersensitivity, demonstrating a variable spectrum of cutaneous and systemic involvement, are reported under various names, especially drug reaction with eosinophilia and systemic symptoms (DRESS). Case definition and overlap with other severe cutaneous adverse reactions (SCAR) are debated. ObjectivesTo analyse the spectrum of signs and symptoms of DRESS and distribution of causative drugs in a large multicentre series. Patients and methodsRegiSCAR, a multinational registry of SCAR, prospectively enrolled 201 potential cases from 2003 to mid-2009. Using a standardized scoring system, 117 cases were validated as showing probable or definite DRESS. ResultsThe male/female ratio was 080; females were borderline significantly younger than males. Next to the ubiquitous exanthema, the main features were eosinophilia (95%), visceral involvement (91%), high fever (90%), atypical lymphocytes (67%), mild mucosal involvement (56%) and lymphadenopathy (54%). The reaction was protracted in all but two patients; two patients died during the acute phase. Drug causality was plausible in 88% of cases. Antiepileptic drugs were involved in 35%, allopurinol in 18%, antimicrobial sulfonamides and dapsone in 12% and other antibiotics in 11%. The median time interval after drug intake was 22days (interquartile range 17-31) for all drugs with (very) probable causality, with differences between drugs. ConclusionThis prospective observational study supports the hypothesis that DRESS is an original phenotype among SCAR in terms of clinical and biological characteristics, causative drugs, and time relation. The diversity of causative drugs was rather limited, and mortality was lower than that suggested by prior publications.
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TL;DR: Physicians' knowledge is essential to the improvement of diagnosis and management, and in the limitation and prevention of long-term sequelae.
289 citations
TL;DR: In this paper, a comprehensive literature search identified 95 articles with 202 cases of M pneumoniae-associated mucocutaneous disease, and the authors suggested the term "Mycoplasma-induced rash and mucositis" for these cases.
Abstract: Background Mycoplasma pneumoniae infection is associated with extrapulmonary complications, including mucocutaneous eruptions. These eruptions, which have been termed either "Stevens-Johnson syndrome" or "erythema multiforme" in the literature, may differ from drug-induced Stevens-Johnson syndrome or viral-associated erythema multiforme. Objective We sought to review the literature characterizing morphology and disease course of M pneumoniae-associated mucocutaneous disease. Methods A comprehensive literature search identified 95 articles with 202 cases. Results Patients were often young (mean age: 11.9 years) and male (66%). Cutaneous involvement ranged from absent (34%), to sparse (47%), to moderate (19%). Oral, ocular, and urogenital mucositis was reported in 94%, 82%, and 63% of cases, respectively. Treatments included antibiotics (80%), systemic corticosteroids (35%), supportive care alone (8%), and/or intravenous immunoglobulin (8%). Complications included mucosal damage (10%), cutaneous scarring (5.6%), recurrence (8%), and mortality (3%). Limitations Mild cases may not have been published; thus this review may have a bias toward more severe disease. Conclusion M pneumoniae-associated mucocutaneous disease has prominent mucositis and sparse cutaneous involvement, although cutaneous involvement varies. Because of the distinct morphology, mild disease course, and potentially important clinical implications regarding treatment, we propose a revision of the nomenclature system and suggest the term "Mycoplasma-induced rash and mucositis" for these cases.
229 citations
01 Jan 2014
TL;DR: In this article, the authors proposed a method to improve the quality of the information provided by the user by using the information from the user's profile and the user profile of the service provider.
Abstract: Натрийуретические пептиды (НУП) являются важными биомаркерами в диагностике и определении прогноза у пациентов с сердечной недостаточностью (СН). Оценка динамики концентрации НУП (BNP, Nt -proBNP) может быть использована в качестве критерия успешности проводимой терапии. так, при достижении целевых уровней НУП можно прогнозировать благоприятный исход заболевания. В настоящее время лечение СН с учетом уровней НУП является частью рекомендаций по лечению СН (класс IIа) и улучшению ее исхода (класс IIб) в США, однако такой подход не используется в российских клиниках. Цель. Представить современный взгляд на возможность использования НУП для оценки эффективности проводимой терапии пациентов с СН. Ключевые слова: натрийуретические пептиды, сердечная недостаточность, оценка эффективности терапии.
167 citations
TL;DR: An updated overview of drug-induced hypersensitivity syndrome (DiHS)/drug reaction with eosinophilia and systemic symptoms (DRESS) is provided and a scoring system is established that can be used to monitor severity, predict prognosis and stratify the risk of developing severe complications including fatal cytomegalovirus disease.
163 citations
TL;DR: An overview of DRESS syndrome is provided, including clinical presentations, histopathological features, pathomechanisms, and treatments, as well as emerging studies have outlined the disease more clearly.
Abstract: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe multiorgan hypersensitivity reaction mostly caused by a limited number of eliciting drugs in patients with a genetic predisposition. Patients with DRESS syndrome present with characteristic but variable clinical and pathological features. Reactivation of human herpesviruses (HHV), especially HHV-6, is the hallmark of the disease. Anti-viral immune responses intertwined with drug hypersensitivity make the disease more complicated and protracted. In recent years, emerging studies have outlined the disease more clearly, though several important questions remain unresolved. In this review, we provide an overview of DRESS syndrome, including clinical presentations, histopathological features, pathomechanisms, and treatments.
157 citations
Cites background from "Drug reaction with eosinophilia and..."
...In addition, lymphadenopathy can be found in 54% of patients by physical examinations or image studies [14]....
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...Facial edema, which can be found in 76% of patients, is the hallmark feature of the disease [14]....
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...Liver injury is the most common type of organ damage and has been found in 75–94% of patients [2,11,14]....
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...Upon examination, atypical lymphocytosis in the blood can be identified in 27–67% of patients [2,11,14]....
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...The involvement of other organs is occasionally encountered, including the pancreas, gastrointestinal tract, and spleen [11,14,18]....
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References
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TL;DR: This study suggests that an illustrated atlas is a useful tool for standardizing the diagnosis of acute severe bullous disorders that are attributed to drugs or infectious agents.
Abstract: • Background and Design.— To conduct a prospective case-control study about causative factors of severe bullous erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis, we needed to define criteria for classifying the cases and standardize the collection of data so that cases could be reliably diagnosed according to this classification. Based on review of case histories and photographs of patients, a group of experts proposed a classification based on the pattern of erythema multiforme—like lesions (categorized as typical targets, raised or flat atypical targets, and purpuric macules) and on the extent of epidermal detachment. An atlas illustrating this classification that included photographs and schematic drawings was developed. We compared the evaluations of 28 cases by four nonphysicians relying on the atlas with the evaluations of the same cases by five experts not using the atlas to determine the usefulness of this atlas for classifying cases according to our nosologic schema. Results.— The following consensus classification in five categories was proposed: bullous erythema multiforme , detachment below 10% of the body surface area plus localized "typical targets" or "raised atypical targets"; Stevens-Johnson syndrome , detachment below 10% of the body surface area plus widespread erythematous or purpuric macules or flat atypical targets; overlap Stevens-Johnson syndrome— toxic epidermal necrolysis , detachment between 10% and 30% of the body surface area plus widespread purpuric macules or flat atypical targets; toxic epidermal necrolysis with spots , detachment above 30% of the body surface area plus widespread purpuric macules or flat atypical targets; and toxic epidermal necrolysis without spots , detachment above 10% of the body surface area with large epidermal sheets and without any purpuric macule or target. Using the atlas, the nonexperts showed excellent agreement with the experts. Conclusion.— This study suggests that an illustrated atlas is a useful tool for standardizing the diagnosis of acute severe bullous disorders that are attibuted to drugs or infectious agents. Whether the five categories proposed represent distinct etiopathologic entities will require further epidemiologic and laboratory investigations. ( Arch Dermatol. 1993;129:92-96)
1,449 citations
TL;DR: Adverse cutaneous reactions to drugs are frequent, affecting 2 to 3 percent of hospitalized patients, and prompt withdrawal of the offending drug is often the most important action to minimize morbidity.
Abstract: Although the rate of acute severe adverse cutaneous reactions to medications is low, these reactions can affect anyone who takes medications and can result in death or disability1. Even a small number of cases associated with a particular drug may alter the recommendations for its use2–4. Prompt differentiation of severe adverse cutaneous reactions from less serious skin disorders may be difficult. Rapid recognition of severe reactions is essential. Prompt withdrawal of the offending drug is often the most important action to minimize morbidity. Adverse cutaneous reactions to drugs are frequent, affecting 2 to 3 percent of hospitalized . . .
1,425 citations
TL;DR: Risks were increased for trimethoprim–sulfamethoxazole and other sulfonamide antibiotics, chlormezanone, quinolones, and aminopenicillins among drugs usually used for short periods.
Abstract: Background Toxic epidermal necrolysis and Stevens–Johnson syndrome are rare, life-threatening, drug-induced cutaneous reactions. We conducted a case–control study to quantify the risks associated with the use of specific drugs. Methods Data were obtained through surveillance networks in France, Germany, Italy, and Portugal. Drug use before the onset of disease was compared in 245 people who were hospitalized because of toxic epidermal necrolysis or Stevens–Johnson syndrome and 1147 patients hospitalized for other reasons (controls). Crude relative risks were calculated and adjusted for confounding by multivariate methods when numbers were large enough. Results Among drugs usually used for short periods, the risks were increased for trimethoprim–sulfamethoxazole and other sulfonamide antibiotics (crude relative risk, 172; 95 percent confidence interval, 75 to 396), chlormezanone (crude relative risk, 62; 21 to 188), aminopenicillins (multivariate relative risk, 6.7; 2.5 to 18), quinolones (multivariate rel...
1,219 citations
TL;DR: The results indicated that allopurinol-SCAR is strongly associated with a genetic predisposition in Han Chinese, and in particular, HLA-B*5801 allele is an important genetic risk factor for this life-threatening condition.
Abstract: Allopurinol, a commonly prescribed medication for gout and hyperuricemia, is a frequent cause of severe cutaneous adverse reactions (SCAR), which include the drug hypersensitivity syndrome, Stevens–Johnson syndrome, and toxic epidermal necrolysis. The adverse events are unpredictable and carry significant morbidity and mortality. To identify genetic markers for allopurinol–SCAR, we carried out a case-control association study. We enrolled 51 patients with allopurinol–SCAR and 228 control individuals (135 allopurinol-tolerant subjects and 93 healthy subjects from the general population), and genotyped for 823 SNPs in genes related to drug metabolism and immune response. The initial screen revealed strong association between allopurinol–SCAR and SNPs in the MHC region, including BAT3 (encoding HLA-B associated transcript 3), MSH5 (mutS homolog 5), and MICB (MHC class I polypeptide-related sequence B) (P < 10–7). We then determined the alleles of HLA loci A, B, C, and DRB1. The HLA-B*5801 allele was present in all (100%) 51 patients with allopurinol–SCAR, but only in 20 (15%) of 135 tolerant patients [odds ratio 580.3 (95% confidence interval, 34.4–9780.9); corrected P value = 4.7 × 10–24] and in 19 (20%) of 93 of healthy subjects [393.51 (23.23–6665.26); corrected P value = 8.1 × 10–18]. HLA alleles A*3303, Cw*0302, and DRB1*0301 were in linkage disequilibrium and formed an extended haplotype with HLA-B*5801. Our results indicated that allopurinol–SCAR is strongly associated with a genetic predisposition in Han Chinese. In particular, HLA-B*5801 allele is an important genetic risk factor for this life-threatening condition.
1,060 citations
TL;DR: The term of DRESS (Drug Rash with Eosinophilia and Systemic Symptoms) is proposed, to decrease the ambiguity of the denomination of hypersensitivity syndrome.
Abstract: Since the first description by Saltzstein in 1959, the denomination of drug-induced pseudolymphoma was used to describe two cutaneous adverse drug reactions with a histological picture mimicking malignant lymphoma. On the basis of clinical presentation, this term includes two different patterns: (1) hypersensitivity syndrome which begins acutely in the first 2 months after the initiation of the drug and associates fever, a severe skin disease with characteristic infiltrated papules and facial edema or an exfoliative dermatitis, lymphadenopathy, hematologic abnormalities (hypereosinophilia, atypical lymphocytes) and organ involvement such as hepatitis, carditis, interstitial nephritis, or interstitial pneumonitis. The cutaneous histological pattern shows a lymphocytic infiltrate, sometimes mimicking a cutaneous lymphoma, and the mortality rate is about 10%. When organ involvement exists, corticosteroids are often prescribed with dramatic improvement. Relapses may occur. (2) drug-induced pseudolymphoma which has a more insidious beginning with nodules and infiltrated plaques appearing several weeks after the beginning of the drug without constitutional symptoms. A pseudolymphoma pattern is seen on cutaneous histological slides. Complete improvement is usual after drug withdrawal, but a delayed lymphoma is possible. To decrease the ambiguity of the denomination of hypersensitivity syndrome, we propose the term of DRESS (Drug Rash with Eosinophilia and Systemic Symptoms).
849 citations