Epidermal necrolysis: characterization of different phenotypes using an unsupervised clustering analysis

knowledge, this is the largest multicentre evaluation of newly proposed diagnostic criteria, Delphi and PARACELSUS, for ulcerative PG. Though it may be tempting to rely on histopathology, evaluating differential diagnoses, erythematous/violaceous border, irregular shape, associated systemic diseases and response to immunosuppressants may be the most critical clues for differentiating PG from its mimickers. Skin biopsy risks exacerbating disease, but histopathology can be informative when clinical features are not definitive. Though further prospective studies are warranted to investigate the role of novel diagnostic criteria and histopathology, our study concurs with a previous study that PARACELSUS might outperform Delphi in a real-world setting.

[1]  T. Hajar,et al.  Comparison of Three Diagnostic Frameworks for Pyoderma Gangrenosum. , 2020, The Journal of investigative dermatology.

[2]  P. Wolkenstein,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 , 2019, The British journal of dermatology.

[3]  M. Shinohara,et al.  Utility of Skin Biopsy and Culture in the Diagnosis and Classification of Chronic Ulcers: A Single-Institution, Retrospective Study , 2019, The American Journal of dermatopathology.

[4]  D. Margolis,et al.  Development and Validation of a Risk Prediction Model for In-Hospital Mortality Among Patients With Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis—ABCD-10 , 2019, JAMA dermatology.

[5]  S. Benson,et al.  The PARACELSUS score: a novel diagnostic tool for pyoderma gangrenosum , 2019, The British journal of dermatology.

[6]  A. Mostaghimi,et al.  The validity of the diagnostic code for pyoderma gangrenosum in an electronic database , 2018, The British journal of dermatology.

[7]  C. Bodemer,et al.  Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins) , 2018, Orphanet Journal of Rare Diseases.

[8]  Rosie Qin,et al.  Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts , 2018, JAMA dermatology.

[9]  P. Wolkenstein,et al.  Severe cutaneous adverse reactions to drugs , 2017, The Lancet.

[10]  J. Josse,et al.  missMDA: A Package for Handling Missing Values in Multivariate Data Analysis , 2016 .

[11]  A. Qureshi,et al.  Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients , 2011, The British journal of dermatology.

[12]  Sébastien Lê,et al.  FactoMineR: An R Package for Multivariate Analysis , 2008 .

[13]  R. Weenig,et al.  Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria , 2004, International journal of dermatology.

[14]  P. R. Dahl,et al.  Skin ulcers misdiagnosed as pyoderma gangrenosum. , 2002, The New England journal of medicine.