Pustular psoriasis of pregnancy: Clinical and genetic characteristics in a series of eight patients and review of the literature

Dermatologic TherapyVolume 35, Issue 8 e15593 ORIGINAL ARTICLE Pustular psoriasis of pregnancy: Clinical and genetic characteristics in a series of eight patients and review of the literature Mariem Ennouri, Corresponding Author Mariem Ennouri [email protected] orcid.org/0000-0002-3647-3489 Laboratory of Molecular and Functional Genetics, Faculty of Sciences of Sfax, Sfax University, Sfax, Tunisia Correspondence Ennouri Mariem, Laboratory of Molecular and Functional Genetics, Faculty of Sciences of Sfax, Sfax University, Street of Soukra km 4, BP 1171-3000 Sfax, Tunisia. Email: [email protected]Search for more papers by this authorEmna Bahloul, Emna Bahloul Department of Dermatology, CHU Hedi Chaker, Sfax, Sfax University, Sfax, TunisiaSearch for more papers by this authorKhadija Sellami, Khadija Sellami Department of Dermatology, CHU Hedi Chaker, Sfax, Sfax University, Sfax, TunisiaSearch for more papers by this authorSlaheddine Marrakchi, Slaheddine Marrakchi Department of Dermatology, CHU Hedi Chaker, Sfax, Sfax University, Sfax, TunisiaSearch for more papers by this authorFaiza Fakhfakh, Faiza Fakhfakh Laboratory of Molecular and Functional Genetics, Faculty of Sciences of Sfax, Sfax University, Sfax, TunisiaSearch for more papers by this authorHamida Turki, Hamida Turki Department of Dermatology, CHU Hedi Chaker, Sfax, Sfax University, Sfax, TunisiaSearch for more papers by this authorNoura Bougacha-Elleuch, Noura Bougacha-Elleuch Laboratory of Molecular and Functional Genetics, Faculty of Sciences of Sfax, Sfax University, Sfax, TunisiaSearch for more papers by this author Mariem Ennouri, Corresponding Author Mariem Ennouri [email protected] orcid.org/0000-0002-3647-3489 Laboratory of Molecular and Functional Genetics, Faculty of Sciences of Sfax, Sfax University, Sfax, Tunisia Correspondence Ennouri Mariem, Laboratory of Molecular and Functional Genetics, Faculty of Sciences of Sfax, Sfax University, Street of Soukra km 4, BP 1171-3000 Sfax, Tunisia. Email: [email protected]Search for more papers by this authorEmna Bahloul, Emna Bahloul Department of Dermatology, CHU Hedi Chaker, Sfax, Sfax University, Sfax, TunisiaSearch for more papers by this authorKhadija Sellami, Khadija Sellami Department of Dermatology, CHU Hedi Chaker, Sfax, Sfax University, Sfax, TunisiaSearch for more papers by this authorSlaheddine Marrakchi, Slaheddine Marrakchi Department of Dermatology, CHU Hedi Chaker, Sfax, Sfax University, Sfax, TunisiaSearch for more papers by this authorFaiza Fakhfakh, Faiza Fakhfakh Laboratory of Molecular and Functional Genetics, Faculty of Sciences of Sfax, Sfax University, Sfax, TunisiaSearch for more papers by this authorHamida Turki, Hamida Turki Department of Dermatology, CHU Hedi Chaker, Sfax, Sfax University, Sfax, TunisiaSearch for more papers by this authorNoura Bougacha-Elleuch, Noura Bougacha-Elleuch Laboratory of Molecular and Functional Genetics, Faculty of Sciences of Sfax, Sfax University, Sfax, TunisiaSearch for more papers by this author First published: 22 May 2022 https://doi.org/10.1111/dth.15593 Funding information: Ministry of Higher Education and Scientific Research-Tunisia, Grant/Award Number: LR16E16 Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Abstract Pustular psoriasis of pregnancy (PPP) can lead to life-threatening complications. The objective of this study is to report clinical and genetic spectrum, prognostic factors and management options. A retrospective study was designed including eight PPP patients. Clinical data were collected, and performed genetic and statistical analysis to identify factors associated with fetal complications, resistance to treatment and post-partum flare extension. A systematic review of the literature was also carried out. Eight Tunisian patients, with a mean age of 23 ± 3.3 years, were included. They presented 14 flares (F) during pregnancies and one flare after delivery. Additional GPP flares outside pregnancy periods were noted in 2/8 of patients. The mean duration of PPP flares was 16.66 ± 7.8 weeks. The first flare occurred at a gestational age of 26 ± 5 weeks. Only 2/8 studied patients presented a homozygous mutation c.80 T > C (p.L27P) in IL36RN gene. Used treatments were topical steroids (n = 12F), systemic steroids (n = 5F), ciclosporin (n = 1F), UVB (n = 1F) and acitretin (in post-partum n = 6F). Complications were oligoamnios (n = 2), intra-uterine growth retardation (n = 1), fetal death in utero (n = 1), prematurity (n = 3), low weight at birth (n = 2). A significant association was found between (i) occurrence of fetal complications and early gestational age at the onset (p = 0.036), (ii) resistance to topical steroids and body surface affected area (p = 0.008), (iii) presence of mutation c.80 T > C in PPP flares and low serum levels of calcium (p = 0.01). Our systematic review of the literature identified 39 patients with 41 flares of PPP. Only 7/39 patients presented a causative mutation in IL36RN and CARD14 genes. PPP is characterized by a phenotypic heterogeneity and can be associated to IL36RN mutations. Its early onset can be associated with fetal complications. 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