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Generalized Pustular Psoriasis

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https://www.readbyqxmd.com/read/28108048/bullous-pseudobullous-pustular-dermatoses
#1
Mark R Wick
Several dermatoses are typified by the formation of spaces (blisters; bullae) within or beneath the epidermis. These may be acellular or filled with particular species of inflammatory cells. Etiological categories include infectious, immune-mediated, genetic, drug-related, and idiopathic lesions. Examples of such disorders include impetigo, Herpes virus infections, pemphigus, bullous pemphigoid and pemphigoid gestationis, epidermolysis bullosa acquisita, IgA-related dermatoses, inherited epidermolysis bullosa variants, Hailey-Hailey disease, and porphyria cutanea tarda...
December 14, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28063630/clinical-profiles-of-pediatric-patients-with-gpp-alone-and-with-different-il36rn-genotypes
#2
Yirong Wang, Ruhong Cheng, Zhiyong Lu, Yifeng Guo, Ming Yan, Jianying Liang, Peichen Huang, Ming Li, Zhirong Yao
BACKGROUND: IL36RN mutation has been identified as one pathogenesis of generalized pustular psoriasis, but the existence of GPP patients without mutation makes this controversial. OBJECTIVE: Our study aimed at assessing the differences in clinical profiles of children with GPP, with and without IL36RN mutation. METHODS: An ambispective case series study involved review of the records of 66 childhood patients with pediatric-onset GPP and without previous psoriasis vulgaris...
December 13, 2016: Journal of Dermatological Science
https://www.readbyqxmd.com/read/28043870/il-1-and-il-36-are-dominant-cytokines-in-generalized-pustular-psoriasis
#3
Andrew Johnston, Xianying Xing, Liza Wolterink, Drew H Barnes, ZhiQiang Yin, Laura Reingold, J Michelle Kahlenberg, Paul W Harms, Johann E Gudjonsson
BACKGROUND: Generalized pustular psoriasis (GPP) is a rare, debilitating, and often life-threatening inflammatory disease characterized by episodic infiltration of neutrophils into the skin, pustule development, and systemic inflammation, which can manifest in the presence or absence of chronic plaque psoriasis (PV). Current treatments are unsatisfactory warranting a better understanding of GPP pathogenesis. OBJECTIVE: To understand better the disease mechanism of GPP to allow improved targeted therapies...
December 30, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27990381/psoriasis-epidemiology-clinical-features-co-morbidities-and-clinical-scoring
#4
Sunil Dogra, Rahul Mahajan
On the basis of current evidence derived from hospital-based studies, mostly from North India, the prevalence of psoriasis in adults varies from 0.44 to 2.8%, with a much lower prevalence in children. The peak age at onset in adults is in the third and fourth decade of life, with a slight male preponderance. It is recommended that population-based large epidemiologic studies should be undertaken in different parts of the country for estimating the correct prevalence of psoriasis in general population. Chronic plaque-type psoriasis is the most common morphologic presentation of psoriasis, accounting for more than 90% of all cases...
November 2016: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/27915962/generalized-pustular-psoriasis-von-zumbusch-type-following-renal-transplant-a-case-report
#5
Georgia Dedemadi, Maria Koukoulaki, Vassileios Vougas, Constantinos Noutsis, Aikaterini Pantelidaki, Spiros Drakopoulos
Generalized pustular psoriasis appears as an uncommon variant form of psoriasis consisting of widespread pustules on an erythematous background (von Zumbusch). A 39-year-old male patient with a history of plaque psoriasis since the age of 9 who had an acute relapse of generalized pustular psoriasis 12 days following a successful renal transplantation is presented. Despite administered immunosuppression for transplantation, the addition of cyclosporine and methotrexate did not reverse the ongoing process of disease and the patient died on the 57th posttransplant day due to multiorgan failure subsequent to severe bone marrow suppression...
December 2, 2016: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/27904292/a-case-of-acrodermatitis-continua-accompanying-with-osteolysis-and-atrophy-of-the-distal-phalanx-that-evoluted-into-generalized-pustular-psoriasis
#6
Kyung Ho Kim, Hong Lim Kim, Hyun Yi Suh, Jae Wook Jeon, Ji Young Ahn, Mi Youn Park, Jai Il Youn
No abstract text is available yet for this article.
December 2016: Annals of Dermatology
https://www.readbyqxmd.com/read/27900482/mutations-in-il36rn-are-associated-with-geographic-tongue
#7
Jianying Liang, Peichen Huang, Huaguo Li, Jia Zhang, Cheng Ni, Yirong Wang, Jinwen Shen, Chunxiao Li, Lu Kang, Jie Chen, Hui Zhang, Zhen Wang, Zhen Zhang, Ming Li, Zhirong Yao
Geographic tongue (GT) is a benign inflammatory disorder of unknown etiology. Epidemiology and histopathology in previous studies found that generalized pustular psoriasis (GPP) is a factor associated with GT, but the molecular mechanism remains obscure. To investigate the mechanism of GT, with and without GPP, three cohorts were recruited to conduct genotyping of IL36RN, which is the causative gene of GPP. In a family spanning three generations and diagnosed with only GT ("GT alone"), GT was caused by the c...
November 29, 2016: Human Genetics
https://www.readbyqxmd.com/read/27884600/six-transmembrane-epithelial-antigens-of-the-prostate-comprise-a-novel-inflammatory-nexus-in-patients-with-pustular-skin-disorders
#8
Yun Liang, Xianying Xing, Maria A Beamer, William R Swindell, Mrinal K Sarkar, Liza Wolterink Roberts, John J Voorhees, J Michelle Kahlenberg, Paul W Harms, Andrew Johnston, Johann E Gudjonsson
BACKGROUND: Pustular skin disorders are a category of difficult-to-treat and potentially life-threatening conditions that involve the appearance of neutrophil-rich pustules. The molecular basis of most pustular skin conditions has remained unknown. OBJECTIVE: We sought to investigate the molecular basis of 3 pustular skin disorders: generalized pustular psoriasis (GPP), palmoplantar pustulosis (PPP), and acute generalized exanthematous pustulosis (AGEP). METHODS: Microarray analyses were performed to profile genome-wide gene expression of skin biopsy specimens obtained from patients with GPP, PPP, or AGEP and healthy control subjects...
November 21, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27828647/generalized-pustular-psoriasis-induced-by-systemic-steroid-dose-reduction
#9
Danielle Cristine Westphal, Antonio Pedro Mendes Schettini, Petra Pereira de Souza, Jessica Castiel, Carlos Alberto Chirano, Mônica Santos
Generalized pustular psoriasis, or psoriasis of von Zumbusch, is an acute and severe clinical form of psoriasis, which usually occurs in patients with psoriasis undergoing aggravating factors. In this work, we report the case of a female patient, 70 years old, who developed generalized pustular psoriasis symptoms while reducing the dose of oral corticosteroids, improperly introduced for the treatment of alleged acute generalized exanthematous pustulosis. The differential diagnosis of generalized pustular psoriasis should be made with other pustular dermatoses, such as subcorneal pustulosis, IgA pemphigus and especially with acute generalized exanthematous pustulosis...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/27795221/-acute-generalized-exanthematous-pustulosis-induced-by-piroxicam
#10
Ingrid Bissinger, Gustavo Matute-Turizo, María Natalia Mejía-Barreneche
BACKGROUND: Between 62 and 90% of cases of acute generalized exanthematous pustulosis are caused by drugs. Its onset is rapid with generalized pustules, fever, and blood neutrophil count over 7000; pustules resolve spontaneously in less than 15 days. A case associated with piroxicam described. CASE REPORT: A 36-year-old with initial erythema of the thorax and abdomen, accompanied by burning, without fever, which later spread to his forearms, upper arms, and thighs, with face edema...
October 2016: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/27771982/systemic-cyclosporine-treatment-in-severe-childhood-psoriasis-a-retrospective-chart-review
#11
Sunil Dogra, Rahul Mahajan, Tarun Narang, Sanjeev Handa
BACKGROUND: Data regarding the use of cyclosporine (CYC) in the treatment of childhood psoriasis is meager. MATERIALS AND METHODS: The records of all psoriasis patients aged less than 18 years and treated with systemic CYC at our institute were retrieved. Clinical status of patients was assessed at regular intervals and response to therapy was graded as good (50-75% decrease in PASI) and excellent (>75% decrease). Laboratory investigations to detect CYC-induced toxicity were done at regular intervals...
October 24, 2016: Journal of Dermatological Treatment
https://www.readbyqxmd.com/read/27743397/adalimumab-administration-after-infliximab-therapy-is-a-successful-treatment-strategy-for-generalized-pustular-psoriasis
#12
Ai Matsumoto, Mayumi Komine, Masaru Karakawa, Megumi Kishimoto, Mamitaro Ohtsuki
We report a case of a 70-year-old woman with generalized pustular psoriasis (GPP) who responded well to infliximab therapy and adalimumab therapy after secondary failure of infliximab therapy, but did not respond to ustekinumab therapy. We speculate that the pathogenic factor in this case favored anti-tumor necrosis factor (TNF)-α therapy to anti-interleukin-12/23 therapy. Herein, we also briefly present three additional cases of treatment with adalimumab after secondary failure of infliximab. GPP is often difficult to treat, and no placebo-controlled trials have been conducted to guide the use of biologics against it because of a paucity of cases...
October 15, 2016: Journal of Dermatology
https://www.readbyqxmd.com/read/27726163/efficacy-and-safety-of-ixekizumab-treatment-for-japanese-patients-with-moderate-to-severe-plaque-psoriasis-erythrodermic-psoriasis-and-generalized-pustular-psoriasis-results-from-a-52-week-open-label-phase-3-study-uncover-j
#13
Hidehisa Saeki, Hidemi Nakagawa, Ko Nakajo, Taeko Ishii, Yoji Morisaki, Takehiro Aoki, Gregory S Cameron, Olawale O Osuntokun
Psoriasis, a chronic, immune-mediated skin disease characterized by red, scaly plaques, affects approximately 0.3% of the population in Japan. The aim of this open-label study was to evaluate the long-term efficacy and safety of ixekizumab, a humanized, anti-interleukin-17A monoclonal antibody, in Japanese patients with plaque psoriasis (n = 78, including 11 psoriatic arthritis), erythrodermic psoriasis (n = 8) and generalized pustular psoriasis (n = 5). Ixekizumab was administrated s.c. at baseline (week 0, 160 mg), from weeks 2 to 12 (80 mg every 2 weeks), and from weeks 16 to 52 (80 mg every 4 weeks)...
October 11, 2016: Journal of Dermatology
https://www.readbyqxmd.com/read/27542682/mutation-analysis-of-il36rn-gene-in-japanese-patients-with-palmoplantar-pustulosis
#14
Toshifumi Takahashi, Noriki Fujimoto, Miho Kabuto, Takeshi Nakanishi, Toshihiro Tanaka
Loss-of-function mutations of the IL36RN gene, encoding interleukin-36 receptor antagonist (IL-36Ra), have been reported as major pathogenic causes of generalized pustular psoriasis (GPP), especially in cases lacking previous histories of psoriasis vulgaris. Palmoplantar pustulosis (PPP), which is traditionally included among GPP-related diseases, has a controversial association with IL36RN. While a negative view about the said association has been recently published from Europe, variations of the IL36RN gene show great ethnic differences...
August 20, 2016: Journal of Dermatology
https://www.readbyqxmd.com/read/27512494/two-case-reports-of-generalized-pustular-psoriasis-of-pregnancy-different-outcomes
#15
REVIEW
Aoibheann Flynn, Naomi Burke, Bridgette Byrne, Noreen Gleeson, Bairbre Wynne, Louise Barnes
Generalized pustular psoriasis of pregnancy is a rare dermatosis with potential serious consequences for both the mother and fetus. Treatment is difficult and historically steroids were the mainstay of treatment. Cyclosporin has been used for a few cases resistant to steroids. We report our own experience of two cases of generalized pustular psoriasis of pregnancy. Cases of generalized pustular psoriasis of pregnancy need review by a dermatologist with experience of skin disorders in pregnancy. Both the fetus and mother need to be monitored closely when systemic illness occurs, as there is a risk of stillbirth...
June 2016: Obstetric Medicine
https://www.readbyqxmd.com/read/27511608/metabolic-syndrome-and-psoriasis-severity-in-south-east-asian-patients-an-investigation-of-potential-association-using-current-and-chronological-assessments
#16
Leena Chularojanamontri, Chanisada Wongpraparut, Narumol Silpa-Archa, Pichanee Chaweekulrat
Although studies regarding prevalence of metabolic syndrome (MS) in Asian psoriatic patients are limited and show varying results, a previous report describes a significant increase in prevalence of MS in Thai psoriatic patients, as compared with rates in the general population. However, no significant association between MS and psoriasis severity using the Psoriasis Area and Severity Index (PASI) was found, which differs from the findings of Korean and Japanese studies. This study aimed at re-evaluating the association between MS and psoriasis severity in Thai patients using current assessment (PASI) and chronological assessment (historical course and interventions)...
December 2016: Journal of Dermatology
https://www.readbyqxmd.com/read/27489427/immunohistochemical-comparison-of-il-36-and-the-il-23-th17-axis-of-generalized-pustular-psoriasis-and-acute-generalized-exanthematous-pustulosis
#17
Hyo Sang Song, Sang Jin Kim, Tae-In Park, Yong Hyun Jang, Eun-So Lee
BACKGROUND: Cutaneous pustular disorders include generalized pustular psoriasis (GPP) and acute generalized exanthematous pustulosis (AGEP). OBJECTIVE: To identify differences between GPP and AGEP, here we immunohistochemically evaluated interleukin (IL)-36 and the IL-23/Th17 axis. METHODS: This retrospective comparative immunohistochemical study was completed using 11 biopsies of 11 cases of GPP and 11 biopsies of 11 cases of AGEP. Through staining with the anti-IL-36-alpha (IL-36α), anti-IL-36 receptor antagonist (IL-36Ra), anti-nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB), anti-IL-23, anti-IL-17, and anti-IL-8 antibodies, main expression location and intensity were visualized in the epidermis and dermis...
August 2016: Annals of Dermatology
https://www.readbyqxmd.com/read/27462478/biologics-and-pediatric-generalized-pustular-psoriasis-an-emerging-therapeutic-trend
#18
REVIEW
Sami K Saikaly, Monica Mattes
Generalized pustular psoriasis (GPP) is a rare form of childhood psoriasis, often requiring systemic therapy, which is challenging as there is a paucity of randomized controlled trials and standardized guidelines. Biologic agents have been used in adults and in pediatric plaque psoriasis, but evidence regarding their efficacy in pediatric GPP has slowly become available. The objective of this study is to summarize and compare the efficacy and safety of biologic agents, such as etanercept, infliximab, and adalimumab, in the treatment of pediatric GPP...
2016: Curēus
https://www.readbyqxmd.com/read/27462226/a-pediatric-case-of-acute-generalized-pustular-eruption-without-streptococcal-infection
#19
Nobuko Tabata, Hideka Yoshizawa
Generalized pustular lesions characterized by acute onset with fever occur in pustulosis acuta generalisata, acute generalized exanthematous pustulosis, and generalized pustular psoriasis. In the present report, we describe a pediatric case of generalized pustular eruption that was not completely consistent with clinical features. Our patient had no evidence of a post-streptococcal infection. We observed scattered symmetric eruption of discrete pustules with an inflammatory halo on normal skin. The eruption was absent on her palms and soles of the feet...
May 2016: Case Reports in Dermatology
https://www.readbyqxmd.com/read/27364786/tumor-necrosis-factor-alpha-induced-protein-3-interacting-protein-1-gene-polymorphisms-and-pustular-psoriasis-in-chinese-han-population
#20
Jian-Wen Han, Yong Wang, Chulu Alateng, Hong-Bin Li, Yun-Hua Bai, Xin-Xiang Lyu, Rina Wu
BACKGROUND: Psoriasis is a common immune-mediated inflammatory dermatosis. Generalized pustular psoriasis (GPP) is the severe and rare type of psoriasis. The association between tumor necrosis factor-alpha induced protein 3 interacting protein 1 (TNIP1) gene and psoriasis was confirmed in people with multiple ethnicities. This study was to investigate the association between TNIP1 gene polymorphisms and pustular psoriasis in Chinese Han population. METHODS: Seventy-three patients with GPP, 67 patients with palmoplantar pustulosis (PPP), and 476 healthy controls were collected from Chinese Han population...
July 5, 2016: Chinese Medical Journal
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