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. 2021 Jul;35(7):1569-1576.
doi: 10.1111/jdv.17274. Epub 2021 Jun 5.

Towards a better understanding of adult idiopathic epidermal necrolysis: a retrospective study of 19 cases

P Monnet  1 , C Rodriguez  2   3   4 , O Gaudin  1   4 , P Cirotteau  5 , B Papouin  6 , O Dereure  4   7 , F Tetart  4   8 , S Lalevee  1   9 , A Colin  1   4 , B Lebrun-Vignes  4   10 , E Abe  11 , J-C Alvarez  11 , V Demontant  2   3 , G Gricourt  2   3 , N de Prost  4   12   13 , C Barau  14 , O Chosidow  1   4   13 , P Wolkenstein  1   4   13 , S Hue  4   9   13 , N Ortonne  4   6   13 , B Milpied  4   5 , S Ingen-Housz-Oro  1   4   15
Affiliations

Towards a better understanding of adult idiopathic epidermal necrolysis: a retrospective study of 19 cases

P Monnet et al. J Eur Acad Dermatol Venereol. 2021 Jul.

Abstract

Background: Most cases of Stevens-Johnson syndrome and toxic epidermal necrolysis are drug-induced. A small subset of cases remain with unknown aetiology (idiopathic epidermal necrolysis [IEN]).

Objective: We sought to better describe adult IEN and understand the aetiology.

Methods: This retrospective study was conducted in 4 centres of the French national reference centre for epidermal necrolysis. Clinical data were collected for the 19 adults hospitalized for IEN between January 2015 and December 2019. Wide toxicology analysis of blood samples was performed. Histology of IEN cases was compared with blinding to skin biopsies of drug-induced EN (DIEN, 'controls'). Available baseline skin biopsies were analysed by shotgun metagenomics and transcriptomics and compared to controls.

Results: IEN cases represented 15.6% of all EN cases in these centres. The median age of patients was 38 (range 16-51) years; 68.4% were women. Overall, 63.2% (n = 12) of cases required intensive care unit admission and 15.8% (n = 3) died at the acute phase. Histology showed the same patterns of early- to late-stage EN with no difference between DIEN and IEN cases. One toxicology analysis showed unexpected traces of carbamazepine; results for other cases were negative. Metagenomics analysis revealed no unexpected pathological microorganism. Transcriptomic analysis highlighted a different pro-apoptotic pathway in IEN compared to DIEN, with an overexpression of apoptosis effectors TWEAK/TRAIL.

Conclusions: IEN affects young people and is a severe form of EN. A large toxicologic investigation is warranted. Different pathways seem involved in IEN and DIEN, leading to the same apoptotic effect, but the primary trigger remains unknown.

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References

    1. Duong TA, Valeyrie-Allanore L, Wolkenstein P, Chosidow O. Severe cutaneous adverse reactions to drugs. Lancet 2017; 390: 1996-2011.
    1. Chaby G, Maldini C, Haddad C et al. Incidence of and mortality from epidermal necrolysis (Stevens-Johnson syndrome/toxic epidermal necrolysis) in France during 2003-16: a four-source capture-recapture estimate. Br J Dermatol 2020; 182: 618-624.
    1. Bettuzzi T, Penso L, de Prost N et al. Trends in mortality rates for Stevens-Johnson syndrome and toxic epidermal necrolysis: experience of a single centre in France between 1997 and 2017. Br J Dermatol 2020; 182: 247-248.
    1. Ingen-Housz-Oro S, Alves A, Colin A et al. Health-related quality of life and long-term sequelae in survivors of epidermal necrolysis: an observational study of 57 patients. Br J Dermatol 2020; 182: 916-926.
    1. Fournier S, Bastuji-Garin S, Mentec H, Revuz J, Roujeau JC. Toxic epidermal necrolysis associated with Mycoplasma pneumoniae infection. Eur J Clin Microbiol Infect Dis 1995; 14: 558-559.