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Generalized Pustular Psoriasis

Megha K Trivedi, Alexandra R Vaughn, Jenny E Murase
Pustular psoriasis of pregnancy (PPP) is a life-threatening condition for both the pregnant mother and fetus, and must be efficiently and accurately diagnosed and treated. This condition has historically been classified as a unique, separate dermatosis of pregnancy. However, current opinion and data suggest that it may be a variant of generalized pustular psoriasis. PPP typically occurs in the third trimester and is characterized by widespread coalescent pustules, desquamation, and systemic symptoms. Clinical features and histopathologic evaluation aid in diagnosis...
2018: International Journal of Women's Health
Dalal Almutairi, Christopher Sheasgreen, Adam Weizman, Afsaneh Alavi
The incidence of anti-tumour necrosis factor (TNF)-induced psoriasiform eruptions has ranged in the literature between 0.5% and 10.9% of patients with inflammatory bowel disease (IBD). The morphology of the rash varies, with plaque psoriasis being the most common form. Generalized pustular eruption is reported in 10.2% of cases of anti-TNF-induced psoriasis. We are reporting a unique case of anti-TNF-associated psoriasiform eruption due to the severity of the rash presenting as a generalized pustular eruption in association with plaque psoriasiform rash requiring systemic therapy...
February 1, 2018: Journal of Cutaneous Medicine and Surgery
Risako Oi, Takuya Takeichi, Yusuke Okuno, Daiei Kojima, Kyoko Sugawara, Michihiro Kono, Hideki Muramatsu, Masashi Akiyama
No abstract text is available yet for this article.
February 6, 2018: Journal of Dermatological Science
Corey Georgesen, Horatio Wildman, Xuan Wang, Cynthia Magro
Pustular psoriasis occurs rarely in children but can present with acute toxicity requiring inpatient admission. For the best approach, medical providers should have an evidence based and systematic treatment algorithm in their armamentarium. Toillustrate this point, we describe a 13-year-old girl who was admitted with generalized pustular psoriasis and who responded dramatically to "wet wrap" therapy and cyclosporine bridged to etanercept. Using this case example, we highlight the three most important considerations in any patient with new onset pustularpsoriasis: avoidance of disease complications, acute "rescue" therapy, and maintenance therapy...
November 15, 2017: Dermatology Online Journal
William R Swindell, Maria A Beamer, Mrinal K Sarkar, Shannon Loftus, Joseph Fullmer, Xianying Xing, Nicole L Ward, Lam C Tsoi, Michelle J Kahlenberg, Yun Liang, Johann E Gudjonsson
IL-36 cytokines have recently emerged as mediators of inflammation in autoimmune conditions including psoriasis vulgaris (PsV) and generalized pustular psoriasis (GPP). This study used RNA-seq to profile the transcriptome of primary epidermal keratinocytes (KCs) treated with IL-1B, IL-36A, IL-36B, or IL-36G. We identified some early IL-1B-specific responses (8 h posttreatment), but nearly all late IL-1B responses were replicated by IL-36 cytokines (24 h posttreatment). Type I and II interferon genes exhibited time-dependent response patterns, with early induction (8 h) followed by no response or repression (24 h)...
2018: Frontiers in Immunology
Masashi Akiyama, Takuya Takeichi, John A McGrath, Kazumitsu Sugiura
Classifying inflammatory skin diseases is challenging, especially for the expanding group of disorders triggered by genetic factors resulting in hyperactivated innate immunity that result in overlapping patterns of dermal and epidermal inflammation with hyperkeratosis. For such conditions, the umbrella term "autoinflammatory keratinization diseases" (AIKD) has been proposed. AIKD encompasses diseases with mixed pathomechanisms of autoinflammation and autoimmunity, and includes IL-36 receptor antagonist (IL-36Ra)-related pustulosis, CARD14-mediated pustular psoriasis, pityriasis rubra pilaris (PRP) type V, and familial keratosis lichenoides chronica (KLC)...
February 1, 2018: Journal of Dermatological Science
Hidenori Ohnishi, Tomonori Kadowaki, Yoko Mizutani, Emi Nishida, Rie Tobita, Namiko Abe, Yukie Yamaguchi, Hikaru Eto, Masaru Honma, Takuro Kanekura, Yukari Okubo, Mariko Seishima, Toshiyuki Fukao, Shigaku Ikeda
No abstract text is available yet for this article.
January 30, 2018: European Journal of Dermatology: EJD
H Bachelez
Patients with pustular psoriasis or related pustular diseases may have genetic abnormalities impairing the function of key players of the innate skin immune system. Recently, identification of these abnormalities has changed the paradigm of several of these diseases. These include generalized pustular psoriasis, palmoplantar pustular psoriasis and acrodermatitis continua of Hallopeau, and also drug-induced acute exanthematous generalized pustular eruption. Identified mutations in IL36RN, CARD14 and AP1S3 in different groups of patients lead to enhanced inflammatory cascade in several cellular subtypes including keratinocytes, and to the recruitment and activation of neutrophils and macrophages...
January 15, 2018: British Journal of Dermatology
Akiko Arakawa, Sigrid Vollmer, Petra Besgen, Adrian Galinski, Burkhard Summer, Yoshio Kawakami, Andreas Wollenberg, Klaus Dornmair, Michael Spannagl, Thomas Ruzicka, Peter Thomas, Jörg C Prinz
Generalized pustular psoriasis (GPP) is the most severe psoriasis variant. Mutations in the IL-36 antagonist IL36RN, in CARD14 or AP1S3 provide genetic evidence for autoinflammatory aetiology but cannot explain its pathogenesis completely. Here we demonstrate that unopposed IL-36 signalling promotes antigen-driven and likely pathogenic T-helper 17 (Th17) responses in GPP. We observed that CD4+ T cells in blood and skin lesions of GPP patients were characterized by intense hyperproliferation, production of the GPP key mediator, IL-17A, and highly restricted T-cell receptor (TCR) repertoires with identical T-cell clones in blood and skin lesions indicating antigen-driven T-cell expansions...
December 27, 2017: Journal of Investigative Dermatology
Mari Orime
Diagnosis of severe cutaneous adverse drug reactions should involve immunohistopathological examination, which gives insight into the pathomechanisms of these disorders. The characteristic histological findings of erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN) provide conclusive evidence demonstrating that SJS/TEN can be distinguished from EM. Established SJS/TEN shows full-thickness, extensive keratinocyte necrosis that develops into subepidermal bullae. Drug-induced hypersensitivity syndrome (DIHS) and exanthema in drug reaction with eosinophilia and systemic symptoms (DRESS) each display a variety of histopathological findings, which may partly correlate with the clinical manifestations...
2017: Journal of Immunology Research
Yoshitaka Ueda, Mayumi Komine, Koji Kamiya, Hidetoshi Tsuda, Takeo Maekawa, Satoru Murata, Mamitaro Ohtsuki
A 92-year-old man developed an erythematous eruption on the trunk and extremities with numerous pustules accompanied by fever. He had never experienced pustular eruption or been diagnosed with psoriasis previously. Skin biopsy revealed Kogoj's spongiform pustule, and he was diagnosed with generalized pustular psoriasis (GPP). Genomic DNA was extracted from his peripheral blood and the sequence of IL36RN gene was analyzed, which revealed a p.Arg10X homozygous mutation. Several cases of elderly-onset GPP have been reported, however, this is the oldest case of GPP...
March 2018: Journal of Dermatology
Qian Wang, Wei Liu, Lixia Zhang
BACKGROUND: Von Zumbusch type of generalized pustular psoriasis is a rare variant of psoriasis in children. It can occur in patients with or without psoriasis vulgaris. OBJECTIVE: The aim of the study was to discuss the precipitating factors, clinical manifestations, laboratory data and therapy of von Zumbusch type of generalized pustular psoriasis in children from southwestern China and to improve the diagnosis and treatment level. METHODS: A retrospective analysis was conducted for inpatients aged 14 years old or less with von Zumbusch type of generalized pustular psoriasis in our department from 2005 to 2014...
May 2017: Anais Brasileiros de Dermatologia
Wei Deng, Chaoyang Miao, Xiaoyan Zhang
We report a case of a 30-year-old Chinese woman with rheumatoid arthritis and interstitial lung disease who abruptly developed generalized pustules and a high fever for 10 days. She had been taking oral prednisone, iguratimod and total glucosides of peony regularly for 5 months prior. In addition, she had taken metronidazole for 3 days 20 days prior which she had used before with no adverse reaction. She had no history of similar lesions and psoriasis. A biopsy of a pustule on the back showed spongiform pustule of Kogoj...
November 23, 2017: Journal of Dermatology
Toshihiro Ito, Hidetoshi Takahashi, Akira Kawada, Hajime Iizuka, Hidemi Nakagawa
Since 1982, the Japanese Society for Psoriasis Research has conducted annual epidemiological surveys of patients with psoriasis. Kawada et al. have reported data for 1982-2001 and Takahashi et al. have reported data for 2002-2008. The present study evaluated 9290 psoriatic cases according to age and sex (2009-2012). The male : female ratio was 2.08:1 (6281 male patients [67.6%] to 3009 female patients [32.4%]). The most prevalent type was psoriasis vulgaris (85.6% of all cases), which was followed by psoriasis arthropathica (6...
November 8, 2017: Journal of Dermatology
Pingjiao Chen, Changxing Li, Rujun Xue, Huiheng Chen, Xin Tian, Kang Zeng, Xibao Zhang, Jingyao Liang
BACKGROUND: There is a few evidence-based information regarding the efficacy and safety of acitretin treatment in children with pustular psoriasis (PP). OBJECTIVE: This study aimed to provide an additional evidence for this field. METHODS: A retrospective study was undertaken for 15 children with PP, received acitretin in doses of 0.6-1.0 mg/kg/day for 4-6 weeks, the transition dose in 0.2-0.4mg/kg/day for 4-6 weeks and maintenance dose of 0...
November 3, 2017: Journal of Dermatological Treatment
Caleb Jeon, Mio Nakamura, Sahil Sekhon, Di Yan, Jashin J Wu, Wilson Liao, Tina Bhutani
No abstract text is available yet for this article.
November 2017: JAAD Case Reports
A E Paniz Mondolfi, M Hernandez Perez, G Blohm, M Marquez, A Mogollon Mendoza, C E Hernandez-Pereira, M A Escalona, A Lodeiro Colatosti, J Rothe DeArocha, A J Rodriguez Morales
Zika virus is an emerging arbovirus, which is expanding in epidemic proportions through tropical and subtropical areas of the world. Although Zika is linked to a number of congenital and neurological complications, there is scarce knowledge on the impact of ZIKV infection in human skin. We report the case of a 68-year old woman who presented with generalized pustular psoriasis after a preceding and otherwise uneventful episode of ZIKV infection. Based on recent experimental data on the biology of ZIKV infection in the cutaneous environment, we speculate that ZIKV may have directly triggered the development of generalized pustular psoriasis by stimulation of keratinocyte-derived mediators of inflammation and a polyfunctional T-cell driven immune reaction in the cutaneous milieu...
October 13, 2017: Clinical and Experimental Dermatology
Cathy Yunjia Zhao, Germana Consuegra, Shaun Chou, Pablo Fernández-Peñas
The introduction of anti-programmed cell death-1 (anti-PD1) monoclonal antibodies has revolutionized the treatment of various advanced malignancies. Despite its efficacy, anti-PD1 therapy is accompanied by a variety of cutaneous adverse events. A 79-year-old man developed erythematous scaly plaques and pustules of the forehead, legs and arms after four cycles of nivolumab infusions every 2 weeks. Histology showed intracorneal pustules with dermal neutrophils and eosinophils. He was treated successfully with topical corticosteroids without discontinuation of nivolumab...
December 2017: Melanoma Research
Ramesh Kumar, Manoj K Sharma, Suresh K Jain, Sumit K Yadav, Anil K Singhal
BACKGROUND: Chikungunya fever is caused by chikungunya virus which is transmitted by the bite of infected Aedes aegypti and A. albopictus mosquitoes. AIMS: To study the various mucocutaneous manifestations in suspected cases of chikungunya fever. MATERIALS AND METHODS: The patients who attended our outpatient department from July 2016 to October 2016 and fulfilled the criteria for "suspect cases" of chikungunya infection stipulated by the National Institute of Communicable Diseases, Directorate General of Health Services, Government of India, were included in the study prospectively...
September 2017: Indian Dermatology Online Journal
R Mössner, D Wilsmann-Theis, V Oji, P Gkogkolou, S Löhr, P Schulz, A Körber, J Christoph-Prinz, R Renner, K Schäkel, L Vogelsang, K-P Peters, S Philipp, K Reich, H Ständer, A Jacobi, A Weyergraf, K Kingo, S Kõks, S Gerdes, K Steinz, T Schill, K G Griewank, M Müller, S Frey, L Ebertsch, S Uebe, M Sticherling, H Sticht, U Hüffmeier
BACKGROUND: Rare variants in the genes IL36RN, CARD14 and AP1S3 have been identified to cause/ contribute to pustular skin diseases, primarily generalized pustular psoriasis (GPP). OBJECTIVES: To better understand the disease-relevance of these genes, we screened our cohorts of patients with pustular skin diseases (primarily GPP and palmoplantar pustular psoriasis [PPP]) for coding changes in these three genes. Carriers of single heterozygous IL36RN mutations were screened for a second mutation in IL36RN...
August 5, 2017: British Journal of Dermatology
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