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

Cabañas R1,2,3,4, Ramírez E3,5, 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 C19, Ortega-Rodríguez NR20, Rivas Becerra B21, Rojas-Perez-Ezquerra P22, Sánchez-González MJ23, Vega-Cabrera C24, 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
6Dermatology Department La Paz University Hospital, Madrid, Spain
7Hospital Universitario y Politécnico la Fe de Valencia, Spain
8Allergy Unit, Hospital Universitario 12 de Octubre, Madrid, Spain
9ARADyAL
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

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

Background: The DRESS syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) is a complex multisystemic severe drug hypersensitivity reaction whose diagnosis and management are troublesome. It involves the management by different specialists. The correct identification of the culprit drug is essential for future safe therapeutical 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 and allergy diagnosis, management treatment and prevention of DRESS syndrome in light of currently available scientific evidence and the experience of multidisciplinary experts.
Methods: These guidelines have been elaborated 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 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. Wherever evidence was lacking, a consensus was reached among the experts.
Results: The first Spanish guidelines on DRESS syndrome are now being published. Important aspects as practical recommendations about clinical diagnosis, identification of the culprit drug through the Spanish pharmacovigilance system algorithm and allergy study have been addressed. Management, treatment and prevention recommendations are provided. Algorithms about management of DRESS in acute and recovery phase have been elaborated. An expert consensus step-wise guidelines for 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