DRESS syndrome: Tricky to diagnose but easy to treat

A 27-year-old female presented to us with a short history of fever, jaundice, rash, and worsening hepatic dysfunction subsequent to treatment with intravenous antibiotics and alternative medicine for a urinary tract infection. The eosinophilia, lymphadenopathy, and transaminitis prompted us to consider a diagnosis of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) which can be fatal if not treated. The patient showed improvement in clinical and laboratory parameters after a course of steroids. This case is presented as DRESS syndrome that can prove rapidly fatal if not diagnosed and treated immediately.

Does sensitization by SARS-CoV-2 immune complexes trigger DRESS syndrome?

Abstract The diagnosis of coronavirus disease (COVID-19) has been a great challenge since the infection affects not only the respiratory system, but also different organs, given the intense inflammatory and autoimmune reaction triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Herein we present a case of a 36-year-old male patient, with some comorbidities and previous use of carbamazepine, who developed a severe condition triggered by COVID-19, including extensive exfoliative erythroderma and severe impairment of liver function, which lasted approximately 80 days.

Predictors of disease severity in drug reaction with eosinophilia and systemic symptoms

Background: Drug reaction with eosinophilia and systemic symptoms is an outcome of a complex interaction between specific drugs, certain herpesviruse types and the immune system of the affected individual and is characterized by an unpredictable course and recurrent flares even after withdrawal of the offending drug and administration of systemic steroids. Aims: To identify the predictors of disease severity in drug reaction with eosinophilia and systemic symptoms. Methods: After obtaining ethical clearance from the institutional ethics committee and a written informed consent from individual study participant, the first hundred patients who required inpatient care in Government Medical College, Kozhikode with drug reaction with eosinophilia and systemic symptoms from January 1st 2011 were included in this study aimed to identify the predictors of disease severity in drug reaction with eosinophilia and systemic symptoms. Results: Male-to-female ratio of the study group was 0.8:1. The presence of atypical cells in peripheral smear and advanced age were found to be predictors of disease severity in drug reaction with eosinophilia and systemic symptoms, whereas, sex, facial erythema and edema and absolute eosinophil count were found not to be predictors of the same. Limitations: The main limitation of this study was our inability to assess the role of human leukocyte antigen (HLA) association and herpes virus reactivation in disease severity in drug reaction with eosinophilia and systemic symptoms. This study was also not designed to evaluate the response to treatment given and the mortality caused by drug reaction with eosinophilia and systemic symptoms. Conclusions: Studies on the predictors of severity in drug reaction with eosinophilia and systemic symptoms in different population groups may enable us to identify the warning signs and help to formulate the standard therapeutic guidelines.

Severe Cutaneous Adverse Reactions: A Single-Center Retrospective Study of 173 Patients in China

BACKGROUND: Severe cutaneous adverse reactions (SCAR) to drugs are a crucial public health issue and the use of systemic corticosteroids in SCAR has been controversial. OBJECTIVE: To analyze clinical features, causative drugs, treatment, outcomes, and prognostic factors of SCAR in the case-series of 173 patients, and add more information to the debate of using systemic corticosteroids in SCAR management. METHODS: A retrospective study of 173 SCAR patients diagnosed with drug reaction with eosinophilia and systemic symptoms (DRESS), Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) or acute generalized exanthematous pustulosis (AGEP) at a tertiary care institution in China between January 2014 and December 2017 was conducted. RESULTS: Of 173 patients, allopurinol, carbamazepine, and antibiotics are the most frequently implicated drugs for DRESS (40.4%), SJS/TEN (26.0%), and AGEP (40.0%) respectively. Moreover, there is a strongly negative correlation between early corticosteroids use and the progression (p=0.000) and severity (p=0.01) of skin lesions. However, there is no association between early corticosteroids use and the mortality of SCAR (odds ratio: 1.01, 95% confidence interval: 0.95~1.08). In addition, lymphadenopathy, eosinophilia, and interval from onset to corticosteroids treatment were correlated with SCAR prognosis. CONCLUSION: Prompt short-course systemic corticosteroids use is associated with early-stage skin lesions remission without influencing the disease mortality. Lymphadenopathy and eosinophilia were the independent poor prognostic factors of SCAR.

Efficacy of combined lymphoplasma exchange for the treatment of severe refractory immune-related skin diseases: a clinical observation / 中华皮肤科杂志

Objective To evaluate the clinical efficacy of lymphoplasma exchange (LPE) for the treatment of severe refractory immune-related skin diseases.Methods From May 2013 to October 2015,8 patients with toxic epidermal necrolysis,drug-induced hypersensitivity syndrome (DIHS),pemphigus vulgaris,pemphigoid or paraneoplastic pemphigus were enrolled from Department of Dermatology,Xiangya Hospital,Central South University,who showed no response to conventional therapy or presented with multiple organ dysfunction.After the treatment with LPE,the efficacy was evaluated,and adverse reactions were observed.Results After one session of LPE therapy,6 patients received marked improvement,and were cured at last.In 1 patient with pemphigus vulgaris who was resistant to the treatment with high doses of glucocorticoids and immunosuppressive agents,the rashes regressed during the treatment with LPE,but recurred after the end of treatment.One patient with bullous pemphigoid presented with eruptive blisters on the next day after the treatment with LPE,which were considered as allergic reactions to allogeneic plasma.There were no obvious differences in white blood cell count,lymphocyte count,neutrophil count and blood platelet count in the peripheral blood of 8 patients before and after the treatment with LPE.During the follow-up of 3-5 years,all of the patients were recovered without recurrence,except 1 patient with bullous pemphigoid who died of disseminated tuberculosis after 1 year.Conclusion LPE is effective for the treatment of severe immune-related skin diseases,but attention should be paid to potential transfusion reaction and allergic reactions.

Síndrome de sensibilidad a fármacos con eosinofilia y síntomas sistémicos en pediatría: Caso clínico / DRESS syndrome in paediatrics: Clinical case

El síndrome de sensibilidad a fármacos con eosinofilia y síntomas sistémicos es una enfermedad potencialmente mortal, caracterizada por exantema, fiebre, adenopatías, alteraciones hematológicas y compromiso de órganos internos. Objetivo: Presentar una afección poco frecuente en pediatría para facilitar la sospecha diagnóstica y el rápido reconocimiento por parte de los médicos. Caso clínico: Lactante de 9 meses hospitalizada por un cuadro de neumonía viral grave con ventilación mecánica no invasiva, tratada con ceftriaxona entre otros medicamentos. Al quinto día de suspendido el antibiótico presentó un exantema maculopapular violáceo, confluente de predominio en el tronco, la cara y las extremidades superiores, asociado a fiebre, eosinofilia y elevación de transaminasas. Se manejó con prednisona oral más corticoides tópicos por 6 semanas, con buena evolución a los 3 meses de seguimiento. Conclusiones: El diagnóstico de síndrome de sensibilidad a fármacos con eosinofilia y síntomas sistémicos se realiza por clínica y exámenes de laboratorio, además de biopsia cutánea en caso de duda diagnóstica. Si bien su causa más frecuente son los anticonvulsivantes se han descrito casos con un sinnúmero de fármacos. El manejo consiste en la suspensión del fármaco sospechoso asociado a medidas de soporte y tratamiento corticosteroide por tiempos prolongados.

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially life-threatening, drug-induced hypersensitivity reaction that includes skin eruption, haematological abnormalities, lymphadenopathy, and internal organ involvement. Objective: Presenting a rare condition in children, to facilitate a rapid diagnostic suspicion and recognition by doctors. Case report: An 9 months old infant admitted due to a severe viral pneumonia, managed with non-invasive ventilation and ceftriaxone. Five days after stopping antibiotics, a confluent maculopapular rash appeared, which was predominantly in the trunk, face and upper extremities, combined with a fever, eosinophilia, and elevated serum levels of transaminase. She received treatment with oral prednisone and topical corticosteroids for 6 weeks, with a good outcome after 3 months. Conclusions: The diagnosis of DRESS syndrome is made using clinical criteria, laboratory values, and histopathology, if there is any query. Although it is classically caused by anticonvulsants and sulphonamides, many other drugs have been implicated. The offending drug should be immediately discontinued and the patient given supportive treatment, and systemic corticosteroids for long periods of treatment.

A 15-year Retrospective Study of DRESS Syndrome: A Single Center Study / 대한피부과학회지

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a syndrome involving multiple organs. Due to a variable clinical presentation and uncertain definition, diagnosis is often delayed or misdiagnosed. OBJECTIVE: The purpose of this study was to investigate the common causative drugs of DRESS and differences according to drugs, clinical features, and prognosis of DRESS, and secondly to compare the differences between steroid use group versus non-use group. METHODS: Medical records of hospitalized patients at the Sanggye Paik Hospital from January 2001 to December 2015 were collected. DRESS patients were enrolled retrospectively using the RegiSCAR diagnostic criteria. RESULTS: A total of 65 patients were included. The four most common causative drug groups were antibiotics (27.7%), anticonvulsants (20%), antituberculosis agents (16.9%), and allopurinol (16.9%). The mean incubation period was 4 weeks, significantly shorter in antibiotics (2 weeks, p < 0.001) and significantly longer in anticonvulsants (6.5 weeks, p=0.033). Sixty-three patients fully recovered with a mean recovery time of 3.1 (standard deviation 2.2) weeks, one patient had sequelae, and one patient died. Recovery time tended to increase with longer duration of diagnosis from rash onset (p < 0.001, correlation coefficient=0.419) and higher serum aspartate aminotransferase levels (p=0.024, correlation coefficient=0.297). The mean recovery time was 1 week shorter for the systemic steroid use group, but it was not statistically significant (p=0.056). CONCLUSION: DRESS may be a heterogeneous syndrome with specific characteristics related to different drugs. The prognosis of DRESS is relatively good and the role of systemic steroid therapy is unclear. Prompt diagnosis and immediate discontinuation of the causative drug are essential for early recovery.

Drug reaction with eosinophilia and systemic symptoms (DRESS): A histopathology based analysis.

Background: The data on the histology of cutaneous lesions of drug reaction with eosinophilia and systemic symptoms (DRESS) is limited. Aims: To study the histopathology of cutaneous lesions of drug reaction with eosinophilia and systemic symptoms (DRESS) and to identify any features with diagnostic or prognostic signifi cance. Methods: All patients admitted to the dermatology ward of government medical college, Kozhikode from January 1, 2014 to December 31, 2014 with probable or defi nite DRESS as per the RegiSCAR scoring system and who were willing to undergo skin biopsy were included in this prospective study. Results: The study population comprised of nine patients. The consistent histological fi nding documented was the predominantly lymphocytic dermal infl ammatory infi ltrate. Four of the fi ve patients whose histology revealed focal interface dermatitis and keratinocyte vacuolation with or without apoptotic keratinocytes, had elevated liver transaminases. Tissue eosinophilia was associated with disease fl ares. The presence of atypical lymphocytes in peripheral smear and histological evidence of dense dermal infl ammatory infi ltrate showed an association with hepatic involvement. Limitations: The main limitations of our study were the small sample size and our inability to carry out a detailed immunohistochemistry work-up. Conclusions: In the appropriate setting, varying combinations of epidermal hyperplasia, spongiosis, parakeratosis and individually necrotic keratinocytes in the background of lymphocyte predominant dermal infi ltrate (with some atypia) favor a diagnosis of drug reaction with eosinophilia and systemic symptoms. Female sex, the presence of atypical lymphocytes in peripheral smear, dense dermal infl ammatory infi ltrate, tissue eosinophilia and interface dermatitis with or without keratinocyte necrosis was associated with a poor prognosis.

Recent advances of pharmacogenomics in severe cutaneous adverse reactions: immune and nonimmune mechanisms

Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and drug reaction with eosinophilia and systemic symptoms (DRESS) are severe cutaneous adverse reactions (SCAR) which are majorly caused by drugs. Though the incidence rate is low, SCAR sometimes can be life-threatening and leads to lifelong sequelae. Many pharmacogenomic associations in immune and nonimmune related genes with the development of SCAR have been discovered recently and the pharmacogenetic tests have been applied to prevent specific drug-induced SCAR. In this review, we discuss the recent advances of pharmacogenomics in SCAR.

Drug reaction with eosinophilia and systemic symptoms: Observations from a tertiary care institution.

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe drug reaction which can mimic a viral infection, an autoimmune disease or a neoplastic disease. Aims: To study the clinical and epidemiological aspects of DRESS and to identify the precipitating drugs. Methods: All patients admitted to the dermatology ward of our tertiary care hospital from 1 st October 2010 to 30 th September 2013 with probable or definite DRESS as per the RegiSCAR scoring system were included in this prospective study. The clinical manifestations observed in the study population were studied and the common offending drugs were identified. Results: During the 3 year study period, 26 patients fulfilled criteria for probable or definite DRESS. In more than 50% of cases, the culprit drug was phenytoin. Most common symptoms observed were fever, rash and facial edema. Liver was the most common internal organ affected. Most of the patients responded to withdrawal of the drug and administration of steroids for 3-6 weeks. One patient with dapsone-induced DRESS died. Conclusions: Intense facial erythema and edema and an elevated eosinophil count were not found to be bad prognostic factors. In most instances the flare ups during the course of the disease could be managed with a slower tapering of steroids. More prospective studies on DRESS are required to assess the prognostic factors and to formulate better diagnostic criteria.

A 5 Year Retrospective Study on the Clinical Patterns and Treatment Outcomes of Severe Cutaneous Adverse Drug Reactions (SCARS) in Hospital Tengku Ampuan Rahimah, Malaysia

Introduction: Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN) , and drug reaction with eosinophilia and systemic symptoms (DRESS) are severe cutaneous adverse drug reactions (SCARs) related to a variety of medications. Objectives: We aim to document the epidemiological features, the causative drugs and clinical outcomes of patients with SCARs treated in Hospital Tengku Ampuan Rahimah (HTAR) between January 2009 and December 2013. Materials & Methods: A retrospective review of the data of all patients with SJS, TEN and DRESS treated from January 2009 to December 2013 was retrieved and analyzed. Results: A total of 33 SCARs patients were seen, which included SJS (25), TEN (3) and DRESS (5). The mean age was 42.8 years. The male-to-female ratio was 1.36:1. Allopurinol (33.3%) was the commonest offending drug, followed by antibiotics (30.3%), anticonvulsants (12.1%), non-steroidal anti-inflammatory drugs (12.1%) and traditional medications (6.1%). Eighty percent of SJS and all TEN and DRESS patients were given systemic corticosteroids. One patient with TEN (33.3%) was concurrently given intravenous immunoglobulin. All SJS patients survived. Two patients with TEN (66.7%) and one patient with DRESS (20%) succumbed due to sepsis. Conclusion: The commonest drugs implicated for SCARs in our study were allopurinol and antibiotics. Inappropriate use of these drugs leads to increased risk of SCARs. Early recognition and prompt treatment of patients with SCARs may improve their outcome.

Propylthiouracil Induced Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) in Graves' Disease / 대한피부과학회지

No abstract available.