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Spanish Guidelines for Diagnosis, Management, Treatment, and Prevention of DRESS Syndrome

Cabañas R1,2,3,4, Ramírez E2,3,5, Sendagorta E3,6, Alamar R7, Barranco R3,8,9, Blanca-López N10, Doña I11,12, Fernández J13, Garcia-Nunez I14, García-Samaniego J15, Lopez-Rico R16, Marín-Serrano E17, Mérida C18, Moya M19, Ortega-Rodríguez NR20, Rivas Becerra B2,21, Rojas-Perez-Ezquerra P3,22, Sánchez- González MJ3,23, Vega-Cabrera C2,24, Vila-Albelda C25, Bellón T2,3,26

1Allergy Unit, Hospital Universitario La Paz, Madrid, Spain
2Institute for Health Research (IdiPAZ), Madrid, Spain
3PIELenRed Consortium
4Centro de Investigación en Red de Enfermedades Raras (CIBERER, U754)
5Pharmacology Department La Paz University Hospital, Madrid, Spain; PIELenRed Consortium
6Dermatology Department La Paz University Hospital
7Hospital Universitario y Politécnico la Fe de Valencia, Valencia, Spain
8Allergy Unit, Hospital Universitario 12 de Octubre, Madrid, Spain
10Allergy Service, Infanta Leonor University Hospital, Madrid, Spain
11Allergy Research Group, Instituto de Investigación Biomédica de Málaga-IBIMA, Hospital Civil, Málaga, Spain
12Allergy Unit, Hospital Regional Universitario de Málaga, Málaga, Spain
13Allergy Section, Alicante University Hospital, ISABIAL-UMH, Alicante, Spain
14Allergy and Pneumology Department, Hospital Quironsalud Campo de Gibraltar, Los Barrios (Cádiz), Spain
15Liver Unit, Hospital Universitario La Paz, CIBERehd, IdiPAZ, Madrid, Spain
16Allergy Department, Hospital Universitario de A Coruña, A Coruña, Spain
17Gastroenterology Department La Paz University Hospital, Madrid, Spain
18Allergy Unit, Hospital Clínico San Cecilio, Granada, Spain
19Allergy Unit, Hospital Universitario Torrecárdenas, Almería, Spain
20Allergy Unit, Hospital Universitario de Gran Canaria Dr. Negrín, Las Palmas de Gran Canaria, Spain
21Nephrology Department, La Paz University Hospital, Madrid, Spain
22Allergy Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain
23Allergy Department, Príncipe de Asturias University Hospital, Alcalá de Henares. Madrid, Spain
24Nephrology Department, La Paz University Hospital, Madrid, Spain
25Allergy Unit, "Severo Ochoa" University Hospital, Leganés, Madrid, Spain
26Drug Hypersensitivity Laboratory, La Paz Hospital, Madrid, Spain

J Investig Allergol Clin Immunol 2020; Vol 30(4) : 229-253
doi: 10.18176/jiaci.0480

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a complex multisystemic severe drug hypersensitivity reaction whose diagnosis and management are troublesome. DRESS syndrome requires management by various specialists. The correct identification of the culprit drug is essential to ensure safe future therapeutic options for the patient. There are no previous Spanish guidelines or consensus statements on DRESS syndrome.
Objective: To draft a review and guidelines on the clinical diagnosis, allergy work-up, management, treatment, and prevention of DRESS syndrome in light of currently available scientific evidence and the experience of experts from multiple disciplines.
Methods: These guidelines were drafted by a panel of allergy specialists from the Drug Allergy Committee of the Spanish Society of Allergy and Clinical Immunology (SEAIC), together with other medical specialists involved in the management of DRESS syndrome and researchers from the PIELenRed consortium. A review was conducted of scientific papers on DRESS syndrome, and the expert panel evaluated the quality of the evidence of the literature and provided grades of recommendation. Whenever evidence was lacking, a consensus was reached among the experts.
Results: The first Spanish guidelines on DRESS syndrome are now being published. Important aspects have been addressed, including practical recommendations about clinical diagnosis, identification of the culprit drug through the Spanish pharmacovigilance system algorithm, and the allergy work-up. Recommendations are provided on management, treatment, and prevention. Algorithms for the management of DRESS in the acute and recovery phases have been drawn up. Expert consensus–based stepwise guidelines for the management and treatment of DRESS syndrome are provided.

Key words: DRESS syndrome, Drug reaction with eosinophilia and systemic symptoms, Drug-induced hypersensitivity syndrome, SCAR, Patch tests, Skin tests, Lymphocyte transformation test, Corticosteroids