Clinical and genetic differences between pustular psoriasis subtypes

Twelves, Sophie and Mostafa, Alshimaa and Dand, Nick and Burri, Elias and Farkas, Katalin and Wilson, Rosemary and Cooper, Hywel L. and Irvine, Alan D. and Oon, Hazel H. and Kingo, Külli and Köks, Sulev and Mrowietz, Ulrich and Puig, Luis and Reynolds, Nick and Tan, Eugene Sern-Ting and Tanew, Adrian and Torz, Kaspar and Trattner, Hannes and Valentine, Mark and Wahie, Shyamal and Warren, Richard B. and Wright, Andrew and Bata-Csörgő, Zsuzsa and Szell, Marta and Griffiths, Christopher E. M. and Burden, A. David and Choon, Siew-Eng and Smith, Catherine H. and Barker, Jonathan N. and Navarini, Alexander A. and Capon, Francesca. (2019) Clinical and genetic differences between pustular psoriasis subtypes. Journal of allergy and clinical immunology, 143 (3). pp. 1021-1026.

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Official URL: https://edoc.unibas.ch/72194/

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The term pustular psoriasis indicates a group of severe skin disorders characterized by eruptions of neutrophil-filled pustules. The disease, which often manifests with concurrent psoriasis vulgaris, can have an acute systemic (generalized pustular psoriasis [GPP]) or chronic localized (palmoplantar pustulosis [PPP] and acrodermatitis continua of Hallopeau [ACH]) presentation. Although mutations have been uncovered in IL36RN and AP1S3, the rarity of the disease has hindered the study of genotype-phenotype correlations.; We sought to characterize the clinical and genetic features of pustular psoriasis through the analysis of an extended patient cohort.; We ascertained a data set of unprecedented size, including 863 unrelated patients (251 with GPP, 560 with PPP, 28 with ACH, and 24 with multiple diagnoses). We undertook mutation screening in 473 cases.; Psoriasis vulgaris concurrence was lowest in PPP (15.8% vs 54.4% in GPP and 46.2% in ACH, P < .0005 for both), whereas the mean age of onset was earliest in GPP (31.0 vs 43.7 years in PPP and 51.8 years in ACH, P < .0001 for both). The percentage of female patients was greater in PPP (77.0%) than in GPP (62.5%; P = 5.8 × 10; -5; ). The same applied to the prevalence of smokers (79.8% vs 28.3%, P < 10; -15; ). Although AP1S3 alleles had similar frequency (0.03-0.05) across disease subtypes, IL36RN mutations were less common in patients with PPP (0.03) than in those with GPP (0.19) and ACH (0.16; P = 1.9 × 10; -14; and .002, respectively). Importantly, IL36RN disease alleles had a dose-dependent effect on age of onset in all forms of pustular psoriasis (P = .003).; The analysis of an unparalleled resource revealed key clinical and genetic differences between patients with PPP and those with GPP.
Faculties and Departments:03 Faculty of Medicine > Bereich Spezialfächer (Klinik) > Dermatologie USB > Dermatologie (Navarini)
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Spezialfächer (Klinik) > Dermatologie USB > Dermatologie (Navarini)
UniBasel Contributors:Navarini, Alexander
Item Type:Article, refereed
Article Subtype:Research Article
Note:Publication type according to Uni Basel Research Database: Journal article
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Last Modified:10 Jul 2020 09:24
Deposited On:10 Jul 2020 09:24

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