Read by QxMD icon Read

Generalized Pustular Psoriasis

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
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
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
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
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
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)...
August 11, 2016: Journal of Dermatology
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
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
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
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
P Weisenseel, D Wilsmann-Theis, C Kahl, K Reich, R Mössner
A number of pustular skin diseases share clinical, pathogenetic, and epidemiological aspects with plaque-type psoriasis, and their classification as a separate clinical entity or as a subtype of psoriasis remains controversial, which is also reflected in the multitude of their names. They include generalized pustular psoriasis with its subtypes, acrodermatitis continua suppurativa (Hallopeau), acute pustulosis palmopantaris, palmoplantar pustular psoriasis, and pustular variants of a mostly TNF-blocker triggered paradoxical psoriasiform dermatitis...
June 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
Sam Polesie, Anette Gente Lidholm
is missing (Short communication).
May 27, 2016: Acta Dermato-venereologica
A Finet, M Viguier, O Chazouillères, F Amatore, C Paul, M A Richard, O Chosidow, H Bachelez, E Sbidian
BACKGROUND: Few epidemiologic data are available regarding biologic liver abnormalities during psoriasis flares. OBJECTIVES: The aim of this study was to assess the prevalence of biological liver test abnormalities (LTA) in a psoriasis population and the risk factors associated with LTA. METHODS: A retrospective cross-sectional study in four hospital dermatology tertiary care centres included patients admitted for severe psoriasis flare between 1st January 2010 and 31st December 2011...
May 26, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Marie Tauber, Elodie Bal, Xue-Yuan Pei, Marine Madrange, Amel Khelil, Houria Sahel, Akila Zenati, Mohamed Makrelouf, Khaled Boubridaa, Amel Chiali, Naima Smahi, Farida Otsmane, Bakar Bouajar, Slaheddine Marrakchi, Hamida Turki, Emmanuelle Bourrat, Manuelle Viguier, Yamina Hamel, Hervé Bachelez, Asma Smahi
Homozygous or compound heterozygous IL36RN gene mutations underlie the pathogenesis of psoriasis-related pustular eruptions including generalized pustular psoriasis, palmoplantar pustular psoriasis, acrodermatitis continua of Hallopeau, and acute generalized exanthematous pustular eruption. We identified two unreported IL36RN homozygous mutations (c.41C>A/p.Ser14X and c.420_426del/p.Gly141MetfsX29) in patients with familial generalized pustular psoriasis. We analyzed the impact of a spectrum of IL36RN mutations on IL-36 receptor antagonist protein by using site-directed mutagenesis and expression in HEK293T cells...
September 2016: Journal of Investigative Dermatology
Winnie Choo
Generalized pustular psoriasis is rare in children, but it can occur and affect an extensive body surface area of a child. Treatment regimens can include medications that are not available in pediatric dosage form. Acitretin is considered one of the treatment options for acute generalized pustular psoriasis in children, but, in Singapore, it is only available as Neotigason capsules. Extemporaneous compounding of the powder content in the capsules was developed for formulating the oral acitretin suspension with a standardized formulation table and compounding process at National Skin Centre...
January 2016: International Journal of Pharmaceutical Compounding
K Yamasaki, H Nakagawa, Y Kubo, K Ootaki
BACKGROUND: A T-helper (Th) cell subset Th17 preferentially produces interleukin (IL)-17 and plays a pivotal role in the pathogenesis of psoriasis. However, the pathological roles of IL-17 cascades in generalized pustular psoriasis (GPP) and psoriatic erythroderma (PsE) have not been well established. OBJECTIVES: To evaluate the efficacy and safety of brodalumab, a human immunoglobulin G2 monoclonal antibody against human IL-17-receptor A (IL-17RA), in Japanese patients with GPP and PsE...
April 23, 2016: British Journal of Dermatology
P Skendros, C Papagoras, I Lefaki, A Giatromanolaki, I Kotsianidis, M Speletas, V Bocly, I Theodorou, V Dalla, K Ritis
Generalized pustular psoriasis (GPP) is a severe type of psoriasis accompanied by systemic and often life-threatening manifestations. The efficacy of the IL-1 antagonist anakinra in cases of GPP underscores the role of IL-1 in disease pathogenesis. We present a case of a middle-aged man who developed an abrupt and severe form of GPP with severe eosinophilia and cholestatic hepatitis. The patient received salvage treatment with a combination of glucocorticoids, hydroxyurea and imatinib, while administration of the IL-1 inhibitor anakinra resulted in remission of hepatitis and a signifigant skin improvement...
April 23, 2016: British Journal of Dermatology
Akiko Arakawa, Thomas Ruzicka, Jörg C Prinz
IMPORTANCE: Generalized pustular psoriasis von Zumbusch type (GPP) is the most severe manifestation of psoriasis. The etiology of GPP is only partially understood, and GPP lacks approved treatments. Loss-of-function mutations in the interleukin 36 (IL-36) receptor antagonist (IL36RN), an inhibitor of innate immune activation in the skin, and therapeutic efficacy of IL-1 blockade in a subset of patients with GPP are viewed as evidence for an autoinflammatory pathogenesis. A pathogenic role of T cells has not been considered...
July 1, 2016: JAMA Dermatology
Kelly C Pearson, Dean S Morrell, Susan R Runge, Puneet Jolly
Acute generalized exanthematous pustulosis (AGEP) is a rare cutaneous eruption that often is a reaction to medications, most commonly antibiotics. Clinically, AGEP closely mimics pustular psoriasis and also is similar to subcorneal pustular dermatosis and IgA pemphigus. For clinicians, it is important to differentiate AGEP from pustular psoriasis. Acute generalized exanthematous pustulosis will have an acute drug association. Few cases have been known to be caused by hydroxychloroquine (HCQ). Proper therapeutic management of AGEP includes withdrawal of the offending agent, and resolution typically occurs within 15 days...
March 2016: Cutis; Cutaneous Medicine for the Practitioner
Marina Eskin-Schwartz, Lina Basel-Vanagaite, Michael David, Irina Lagovsky, Dan Ben-Amitai, Pola Smirin-Yosef, Lihi Atzmony, Emmilia Hodak
Psoriasis is a multifactorial chronic inflammatory disease. Monogenic psoriasis has been described recently, including dominantly inherited plaque and generalized pustular types, related to activating mutations in the CARD14 gene. We describe here a family with CARD14-related psoriasis, exhibiting an extreme variability of clinical presentation (from mild plaque-type to generalized pustular psoriasis) and early disease onset. The affected family members harboured the c.349G>A [p.Gly117Ser] mutation in CARD14, which has not previously been linked to pustular psoriatic phenotype...
March 17, 2016: Acta Dermato-venereologica
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"