Management of Adult Patients With Drug Reaction With Eosinophilia and Systemic Symptoms

Author:

Brüggen Marie-Charlotte1234,Walsh Sarah45,Ameri M. Milad123,Anasiewicz Natalie1,Maverakis Emanual6,French Lars E.478,Ingen-Housz-Oro Saskia491011,Abe Richiiro12,Ardern-Jones Michael13,Assier Haudrey9,Barbaud Annick14,Bensaid Benoit15,Bernal William16,Bernier Claire17,Brassard Alain6,Březinová Eva418,Cabañas Rosario192021,Cardones Adela22,Chu Chia-Yu23,Chua Ser-Ling424,Descamps Vincent25,Didona Biagio426,Divito Sherrie Jill27,Dodiuk-Gad Roni282930,Elman Scott31,Gaspar Krisztian32,Mortz Charlotte G.33,Hama Natsumi12,Lee Haur Yueh34,Horváth Barbara35,Jörg Lukas36,Kaffenberger Benjamin H.37,Kucinskiene Vesta438,Lebrun-Vignes Bénédicte39,Lehloenya Rannakoe J.40,Meyersburg Damian441,Micheletti Robert4243,Milpied Brigitte41044,Miyagawa Fumi45,Mostaghimi Arash27,Nägeli Mirjam1,Naldi Luigi46,Oppel Eva7,Phillips Elizabeth J.47,Pirani Tasneem16,Ranki Annamari448,Mälkönen Tarja48,Rosenbach Misha42,Salavastru Carmen440,Staumont-Salle Delphine49,Sandberg Heidi48,Setterfield Jane50,Shinkai Kanade2451,Shiohara Tetsuo52,Soria Angele53,Tartar Danielle6,Tiplica George-Sorin454,Traidl Stephan155,Vorobyev Artem56,von Wachter Camilla2,Worswick Scott57,Cho Yung-Tsu23,

Affiliation:

1. Department of Dermatology, University Hospital of Zurich, Zurich, Switzerland

2. Faculty of Medicine, University of Zurich, Zurich, Switzerland

3. Christine Kühne-Center for Allergy Research and Education (CK-CARE), Davos, Switzerland

4. ToxiTEN group, European Reference Network for Rare Skin Diseases (ERN-Skin), Paris, France

5. Department of Dermatology, King’s College Hospital, London, England, United Kingdom

6. Department of Dermatology, University of California, Davis, Sacramento, California

7. Department of Dermatology and Allergy, University Hospital, Ludwig-Maximilians-University (LMU) Munich, Munich, Germany

8. Dr Phillip Frost Department of Dermatology and Cutaneous Surgery, Miller School of Medicine, University of Miami, Miami, Florida

9. Department of Dermatology, Henri Mondor University Hospital, University of Paris-Est Créteil, Créteil, France

10. Toxic Bullous Dermatoses TOXIBUL Reference Center, Filière FIMARAD, AP-HP, Henri Mondor Hospital, Créteil, France

11. Univ Paris Est Créteil EpiDermE, Créteil, France

12. Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan

13. Department of Dermatology, Clinical Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, England, United Kingdom

14. Sorbonne Université, INSERM, Institut Pierre Louis d’Epidemiologie et de Sante Publique, AP-HP Sorbonne Universite, Tenon Hospital, Department of Dermatology and Allergology, Paris, France

15. Department of Dermatology, Lyon Natecia Hospital, Lyon, France

16. Liver Intensive Therapy Unit, King’s Critical Care, King’s College Hospital, London, England, United Kingdom

17. Department of Dermatology, University Hospital Nantes, Nantes, France

18. Department of Dermatovenereology, St Anne’s Faculty Hospital, Faculty of Medicine, Masaryk University, Brno, Czech Republic

19. Department of Allergy, La Paz University Hospital, IdiPAZ, Madrid, Spain

20. PIELenRed Consortium, Madrid, Spain

21. Center for Biomedical Research Network on Rare Diseases (CIBERER U754), Madrid, Spain

22. Department of Dermatology, University of Kansas, Kansas City

23. Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan

24. Department of Dermatology, University of California, San Francisco

25. Department of Dermatology, Bichat–Claude Bernard Hospital, Paris Diderot University, Paris, France

26. Center of Rare Diseases IDI-IRCCS, Rome, Italy

27. Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts

28. Department of Dermatology, Emek Medical Center, Afula, Israel

29. Division of Dermatology, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada

30. Ruth and Bruce Rappaport Faculty of Medicine, Technion–Israel Institute of Technology, Haifa, Israel

31. Department of Dermatology, Miller School of Medicine, University of Miami, Miami, Florida

32. Department of Dermatology, University of Debrecen, Debrecen, Hungary

33. Department of Dermatology and Allergy Center, Odense University Hospital, University of Southern Denmark, Odense, Denmark

34. Department of Dermatology, Singapore General Hospital, Singapore, Singapore

35. Center for Blistering Diseases, Department of Dermatology, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands

36. Division of Allergology and Clinical Immunology, Department of Pneumology and Allergology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland

37. Department of Dermatology, The Ohio State University, Columbus, Ohio

38. Department of Skin and Venereal Diseases, Lithuanian University of Health Sciences (LUHS), Hospital of LUHS Kauno Klinikos, Kaunas, Lithuania

39. Regional Pharmacovigilance Centre, Pitié-Salpêtrière Hospital, APHP, Sorbonne Université, Paris, France

40. Department of Paediatric Dermatology, Colentina University Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania

41. Department of Dermatology and Allergology, University Hospital Salzburg of the Paracelsus Medical University, Salzburg, Austria

42. Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia

43. Images in Dermatology Editor, JAMA Dermatology

44. Department of Dermatology, University Hospital of Bordeaux, Bordeaux, France

45. Department of Dermatology, Nara Medical University, Kashihara, Japan

46. Department of Dermatology, Ospedale San Bortolo, Vicenza, Italy

47. Department of Medicine and Pharmacology, Vanderbilt University Medical Center, Nashville, Tennessee

48. Department of Skin and Allergic Diseases, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland

49. CHU Lille, Department of Dermatology, Univ Lille, INSERM U1286, Lille Inflammation Translational Research Institute (INFINITE) F-59000, Lille, France

50. Department of Oral Medicine, Guy’s and St Thomas’ NHS Foundation Trust, London, England, United Kingdom

51. Editor, JAMA Dermatology

52. Department of Dermatology, Kyorin University School of Medicine, Mitaka, Japan

53. Department of Dermatology and Allergology, Tenon Hospital, Sorbonne Universite, Paris, France

54. Department of Dermatology II, Colentina Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania

55. Department of Dermatology and Allergy, Hannover Medical School, Hannover, Germany

56. Department of Dermatology, University Hospital Lübeck, Lübeck, Germany

57. Department of Dermatology, Keck School of Medicine, University of Southern California, Los Angeles

Abstract

ImportanceDrug reaction with eosinophilia and systemic symptoms (DRESS) is a rare but potentially fatal drug hypersensitivity reaction. To our knowledge, there is no international consensus on its severity assessment and treatment.ObjectiveTo reach an international, Delphi-based multinational expert consensus on the diagnostic workup, severity assessment, and treatment of patients with DRESS.Design, Setting, and ParticipantsThe Delphi method was used to assess 100 statements related to baseline workup, evaluation of severity, acute phase, and postacute management of DRESS. Fifty-seven international experts in DRESS were invited, and 54 participated in the survey, which took place from July to September 2022.Main Outcomes/MeasuresThe degree of agreement was calculated with the RAND-UCLA Appropriateness Method. Consensus was defined as a statement with a median appropriateness value of 7 or higher (appropriate) and a disagreement index of lower than 1.ResultsIn the first Delphi round, consensus was reached on 82 statements. Thirteen statements were revised and assessed in a second round. A consensus was reached for 93 statements overall. The experts agreed on a set of basic diagnostic workup procedures as well as severity- and organ-specific further investigations. They reached a consensus on severity assessment (mild, moderate, and severe) based on the extent of liver, kidney, and blood involvement and the damage of other organs. The panel agreed on the main lines of DRESS management according to these severity grades. General recommendations were generated on the postacute phase follow-up of patients with DRESS and the allergological workup.Conclusions and RelevanceThis Delphi exercise represents, to our knowledge, the first international expert consensus on diagnostic workup, severity assessment, and management of DRESS. This should support clinicians in the diagnosis and management of DRESS and constitute the basis for development of future guidelines.

Publisher

American Medical Association (AMA)

Subject

Dermatology

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