Toxic epidermal necrolysis, DRESS, AGEP: Do overlap cases exist?

Orphanet Journal of Rare Diseases

Jean-Claude Roujeau³  Pierre Wolkenstein¹  Martine Bagot²  Sylvia H Kardaun⁵  Marie Pauline Konstantinou³  Nicolas Ortonne⁴  Laurence Valeyrie-Allanore¹  Sophie Bouvresse³ 

[1] LIC EA 4393, Henri Mondor Hospital, Assistance Publique Hôpitaux de Paris Université Paris-Est Créteil, Créteil Cedex, F-94010, France;Department of Dermatology, Saint-Louis Hospital, Université Paris VII, Assistance Publique Hôpitaux de Paris, Paris, France;Department of Dermatology, Referral center for toxic and auto-immune blistering diseases, Henri Mondor Hospital, Assistance Publique Hôpitaux de Paris Université Paris-Est Créteil, Créteil Cedex, F-94010, France;Department of Pathology, Henri Mondor Hospital, Assistance Publique Hôpitaux de Paris, Université Paris-Est Créteil, Créteil Cedex, F-94010, France;Department of Dermatology, University Medical Centre Groningen, University of Groningen, Hanzeplein 1, Groningen, 9713 GZ, The Netherlands

关键词: Overlap;    Severe cutaneous adverse reactions;    AGEP;    DRESS;    Toxic epidermal necrolysis;   

Others  :  864198
DOI  :  10.1186/1750-1172-7-72

 received in 2012-04-23, accepted in 2012-09-06,  发布年份 2012

【 摘 要 】

Background

Severe cutaneous adverse reactions to drugs (SCARs) include acute generalized exanthematous pustulosis (AGEP), drug reaction with eosinophilia and systemic symptoms (DRESS) and epidermal necrolysis (Stevens-Johnson syndrome–toxic epidermal necrolysis [SJS-TEN]). Because of the varied initial presentation of such adverse drug reactions, diagnosis may be difficult and suggests overlap among SCARs. Overlapping SCARs are defined as cases fulfilling the criteria for definite or probable diagnosis of at least 2 ADRs according to scoring systems for AGEP, DRESS and SJS-TEN. We aimed to evaluate the prevalence of overlap among SCARs among cases in the referral hospital in France.

Methods

We retrospectively analyzed data for 216 patients hospitalized in the referral centre over 7 years with a discharge diagnosis of AGEP (n = 45), DRESS (n = 47), SJS-TEN (n = 80) or “drug rash” (n = 44). Each case with detailed clinical data and a skin biopsy specimen was scored for AGEP, DRESS and SJS-TEN by use of diagnostic scores elaborated by the RegiSCAR group.

Results

In total, 45 of 216 cases (21%) had at least 2 possible diagnoses: 35 had a single predominant diagnosis (definite or probable), 7 had several possible diagnoses and 3 (2.1% of 145 confirmed SCARs) were overlap SCARs.

Conclusions

Despite ambiguities among SCARs, confirmed overlap cases are rare. This study did not avoid pitfalls linked to its retrospective nature and selection bias. In the acute stage of disease, early identification of severe ADRs can be difficult because of clinical or biologic overlapping features and missing data on histology, biology and evolution. Retrospectively analyzing cases by use of diagnostic algorithms can lead to reliable discrimination among AGEP, DRESS and SJS-TEN.

【 授权许可】

   
2012 Bouvresse et al.; licensee BioMed Central Ltd.

【 图 表 】

【 参考文献 】

• [1]Wolf R, Orion E, Marcos B, Matz H: Life-threatening acute adverse cutaneous drug reactions. Clin Dermatol 2005, 23(2):171-181.
• [2]Roujeau JC, Stern RS: Severe adverse cutaneous reactions to drugs. N Engl J Med 1994, 331(19):1272-1285.
• [3]Valeyrie-Allanore L, Sassolas B, Roujeau JC: Drug-induced skin, nail and hair disorders. Drug Saf 2007, 30(11):1011-1030.
• [4]Sidoroff A, Halevy S, Bavinck JN, Vaillant L, Roujeau JC: Acute generalized exanthematous pustulosis (AGEP)-a clinical reaction pattern. J Cutan Pathol 2001, 28(3):113-119.
• [5]Sidoroff A, Dunant A, Viboud C, et al.: Risk factors for acute generalized exanthematous pustulosis (AGEP) – results of a multinational case–control study (EuroSCAR). Br J Dermatol 2007, 157(5):989-996.
• [6]Bocquet H, Bagot M, Roujeau JC: Drug-induced pseudolymphoma and drug hypersensitivity syndrome (Drug Rash with Eosinophilia and Systemic Symptoms: DRESS). Semin Cutan Med Surg 1996, 15(4):250-257.
• [7]Cacoub P, Musette P, Descamps V, et al.: The DRESS syndrome: a literature review. Am J Med 2011, 124(7):588-597.
• [8]Kardaun SH, Sidoroff A, Valeyrie-Allanore L, Halevy S, Davidovici BB, Mockenhaupt M, Roujeau JC: Variability in the clinical pattern of cutaneous side-effects of drugs with systemic symptoms: does a DRESS syndrome really exist? Br J Dermatol 2007, 156(3):609-611.
• [9]Auquier-Dunant A, Mockenhaupt M, Naldi L, Correia O, Schröder W, Roujeau JC, SCAR Study Group. Severe Cutaneous Adverse Reactions: Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study. Arch Dermatol 2002, 138(8):1019-1024.
• [10]Mockenhaupt M, Viboud C, Dunant A, Naldi L, Halevy S, Bouwes Bavinck JN, Sidoroff A, Schneck J, Roujeau JC, Flahault A: Stevens-Johnson syndrome and toxic epidermal necrolysis: assessment of medication risks with emphasis on recently marketed drugs. The EuroSCAR-study. J Invest Dermatol 2008, 128(1):35-44.
• [11]Valeyrie-Allanore L, Poulalhon N, Fagot JP, Sekula P, Davidovici B, Sidoroff A, Mockenhaupt M: Stevens-Johnson syndrome and toxic epidermal necrolysis induced by amifostine during head and neck radiotherapy. Radiother Oncol 2008, 87(2):300-303.
• [12]Roujeau JC, Bioulac-Sage P, Bourseau C, Guillaume JC, Bernard P, Lok C, Plantin P, Claudy A, Delavierre C, Vaillant L, Wechsler J, Danan G, Bénichou C, Beylot C: Acute generalized exanthematous pustulosis. Analysis of 63 cases. Arch Dermatol 1991, 127(9):1333-1338.
• [13]Speeckaert MM, Speeckaert R, Lambert J, Brochez L: Acute generalized exanthematous pustulosis: an overview of the clinical, immunological and diagnostic concepts. Eur J Dermatol 2010, 20(4):425-433.
• [14]Peermohamed S, Haber RM: Acute generalized exanthematous pustulosis simulating toxic epidermal necrolysis: a case report and review of the literature. Arch Dermatol 2011, 147(6):697-701.
• [15]Machet ML, Vaillant L: Acute generalized exanthematic pustulosis. Ann Dermatol Venereol 2001, 128(1):73-79.
• [16]Kleier RS, Breneman DL, Boiko S: Generalized pustulation as a manifestation of the anticonvulsant hypersensitivity syndrome. Arch Dermatol 1991, 127(9):1361-1364.
• [17]Begon E, Roujeau JC: Drug hypersensitivity syndrome: DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms). Ann Dermatol Venereol 2004, 131(3):293-297.
• [18]Wolf R, Davidovici B, Matz H, Mahlab K, Orion E, Sthoeger ZM: Drug Rash with eosinophilia and systemic symptoms versus Stevens-Johnson Syndrome–a case that indicates a stumbling block in the current classification. Int Arch Allergy Immunol 2006, 141(3):308-310.
• [19]Bombal C, Roujeau JC, Kuentz M, Revuz J, Touraine R: Hematologic anomalies in Lyell’s syndrome. Study of 26 cases. Ann Dermatol Venereol 1983, 110(2):113-119.
• [20]Lebargy F, Wolkenstein P, Gisselbrecht M, Lange F, Fleury-Feith J, Delclaux C, Roupie E, Revuz J, Roujeau JC: Pulmonary complications in toxic epidermal necrolysis: a prospective clinical study. Intensive Care Med 1997, 23(12):1237-1244.
• [21]Blum L, Chosidow O, Rostoker G, Philippon C, Revuz J, Roujeau JC: Renal involvement in toxic epidermal necrolysis. J Am Acad Dermatol 1996, 34(6):1088-1090.
• [22]Valeyrie-Allanore L, Roujeau JC: Denominations and classification of severe cutaneous adverse reactions to drugs: splitters versus mergers. Eur J Dermatol 2007, 17(5):359-360.
• [23]Funck-Brentano E, Duong T, Family D, Bouaziz JD, Ortonne N, Bagot M, Roujeau JC, Wolkenstein P, Valeyrie-Allanore L: Auto-immune thyroiditis and drug reaction with eosinophilia and systemic symptoms (DRESS) associated with HHV-6 viral reactivation. Ann Dermatol Venereol 2011, 138(8–9):580-585.
• [24]Geudry J, Roujeau JC, Binaghi M, Soubrane G, Muraine M: Risk factors for the development of ocular complications of Stevens–Johnson syndrome and toxic epidermal necrolysis. Arch Dermatol 2009, 145(2):157-162.
• [25]Pichler WJ, Naisbitt DJ, Park BK: Immune pathomechanism of drug hypersensitivity reactions. J Allergy Clin Immunol 2011, 127(3):S74-S81.
• [26]Pichler WJ, Adam J, Daubner B, Gentinetta T, Keller M, Yerly D: Drug hypersensitivity reactions: pathomechanism and clinical symptoms. Med Clin North Am 2010, 94(4):645-664.
• [27]Chung WH, Hung SI, Chen YT: Genetic predisposition of life-threatening antiepileptic-induced skin reactions. Expert Opin Drug Saf 2010, 9(1):15-21.
• [28]Picard D, Janela B, Descamps V, D’Incan M, Courville P, Jacquot S, Rogez S, Mardivirin L, Moins-Teisserenc H, Toubert A, Benichou J, Joly P, Musette P: Drug reaction with eosinophilia and systemic symptoms (DRESS): a multiorgan antiviral T cell response. Sci Transl Med 2010, 2(46):46ra62.
• [29]Yawalkar N, Shrikhande M, Hari Y, Nievergelt H, Braathen LR, Pichler WJ: Evidence for a role for IL-5 and eotaxin in activating and recruiting eosinophils in drug-induced cutaneous eruptions. J Allergy Clin Immunol 2000, 106(6):1171-1176.
• [30]Britschgi M, Steiner UC, Schmid S, Depta JP, Senti G, Bircher A, Burkhart C, Yawalkar N, Pichler WJ: T-cell involvement in drug-induced acute generalized exanthematous pustulosis. J Clin Invest 2001, 107(11):1433-1441.
• [31]Sugita K, Kabashima K, Sawada Y, Haruyama S, Yoshioka M, Mori T, Kobayashi M, Ogasawara K, Tokura Y: Increased circulating Th17 frequencies and serum IL-22 levels in patients with acute generalized exanthematous pustulosis. J Eur Acad Dermatol Venereol 2011, 25(4):485-488.
• [32]Pichler WJ: Delayed drug hypersensitivity reactions. Ann Intern Med 2003, 139(8):683-693.
• [33]Roujeau JC: Clinical heterogeneity of drug hypersensitivity. Toxicology 2005, 209(2):123-129.
• [34]Hung SI, Chung WH, Jee SH, Chen WC, Chang YT, Lee WR, Hu SL, Wu MT, Chen GS, Wong TW, Hsiao PF, Chen WH, Shih HY, Fang WH, Wei CY, Lou YH, Huang YL, Lin JJ, Chen YT: Genetic susceptibility to carbamazepine-induced cutaneous adverse drug reactions. Pharmacogenet Genomics 2006, 16(4):297-306.