当前位置： SCI文献检索 > JOURNAL OF DERMATOLOGICAL SCIENCE期刊下所有文献 > Clinical profiles of pediatric patients with GPP alone and with different IL36RN genotypes.

Clinical profiles of pediatric patients with GPP alone and with different IL36RN genotypes.

Abstract：

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. RESULTS:c.115+6T>C was the most common mutation in this Chinese population with GPP alone. The age at onset was nearly halved in the homozygotes/compound heterozygotes than in IL36RN-negative patients. Besides a more severe inflammatory progression, three minor signs could prioritize patients with GPP for IL36RN screening (confluent lakes of pus (P=0.002), perianal erosion (P=0.014), and flexural erosion (P=0.007)). More patients with the pathogenic mutation converted to ACH than those without mutation (χ2=4.773, P=0.029). Children with GPP with or without IL36RN mutation responded well to oral low-dose acitretin, but IL36RN-positive cases suffered a much higher half-year recurrence rate after withdrawl of acitretin treatment(χ2=10.370, P=0.001). CONCLUSIONS:Specific clinical features can remind dermatologists of the necessity of sequencing diagnosis. The mild pustular phenotype of those without mutation may imply the possible role of the epigenetic changes of IL36RN, or other IL36-blockers in the pathogenesis. Pediatric patients with GPP alone, both with and without IL36RN mutation responded well to low-dose acitretin.

journal_name

J Dermatol Sci

journal_title

Journal of dermatological science

authors

Wang Y,Cheng R,Lu Z,Guo Y,Yan M,Liang J,Huang P,Li M,Yao Z

doi

10.1016/j.jdermsci.2016.11.008

subject

Has Abstract

pub_date

2017-03-01 00:00:00

pages

235-240

issue

3

eissn

0923-1811

issn

1873-569X

pii

S0923-1811(16)30958-6

journal_volume

85

pub_type

杂志文章

相关文献

JOURNAL OF DERMATOLOGICAL SCIENCE文献大全
  • Assessment of in vitro percutaneous absorption of glycolic acid through human skin sections using a flow-through diffusion cell system.

    abstract：UNLABELLED:The percutaneous absorption studies were performed using a flow-through diffusion cell system with skin specimens from 24 healthy women to assess the penetration of glycolic acid (GA). Percentages of GA, based on 14C-labelled activity, found in the skin after application of 4% GA at pH 2.0 or pH 3.8, after 2...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/s0923-1811(98)00039-5

    authors： Jiang M,Qureshi SA

    更新日期：1998-12-01 00:00:00

  • Glycolic acid induces keratinocyte proliferation in a skin equivalent model via TRPV1 activation.

    abstract：BACKGROUND:Glycolic acid (GA) is the most commonly used alpha-hydroxy acid (AHA) for dermatologic applications, and is considered as a versatile superficial peeling agent for facial rejuvenation. Its therapeutic effect includes acceleration of epidermal turnover without apparent inflammation, and its action is pH-depen...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/j.jdermsci.2009.11.007

    authors： Denda S,Denda M,Inoue K,Hibino T

    更新日期：2010-02-01 00:00:00

  • Clinical analysis of anti-cardiolipin.beta 2 glycoprotein 1 antibody positive patients in anti-phospholipid syndrome.

    abstract：:Clinical analysis was performed on anti-cardiolipin x beta2 glycoprotein 1 (ACL x beta2 GP1) antibody positive patients with collagen vascular diseases. Nine patients out of 89 showed positive aCL x beta2 GP1 antibody which was a relatively lower percentage compared to that of othoffanti-phospholipid antibodies, such ...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/0923-1811(94)00380-w

    authors： Katayama I,Nishioka K,Otoyama K

    更新日期：1995-05-01 00:00:00

  • Failure to detect paramyxovirus RNA in the skin of connective tissue disease.

    abstract：:Etiology of connective tissue disease is unknown. The association of infectious agents has been suspected serologically, ultrastructurally and recently by means of molecular biological techniques. We extracted RNA from lesions of 25 discoid lupus erythematosus, 9 systemic LE, and 3 systemic sclerosis biopsies, and rev...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/0923-1811(95)00422-x

    authors： Fujiwara H,Fujiwara K,Yotsumoto S,Hashimoto K

    更新日期：1996-01-01 00:00:00

  • Resolvin D1 attenuates imiquimod-induced mice psoriasiform dermatitis through MAPKs and NF-κB pathways.

    abstract：BACKGROUND:Resolvin D1 (RvD1), a pro-resolution lipid mediator derived from docosahexaenoic acid (DHA), has been described to promote several kinds of inflammatory resolution. However, the effects and anti-inflammatory mechanisms of RvD1 on psoriasis have not been previously reported. OBJECTIVE:The present study aimed...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/j.jdermsci.2017.10.016

    authors： Xu J,Duan X,Hu F,Poorun D,Liu X,Wang X,Zhang S,Gan L,He M,Zhu K,Ming Z,Chen H

    更新日期：2018-02-01 00:00:00

  • Sialic acid cyclization of human Th homing receptor glycan associated with recurrent exacerbations of atopic dermatitis.

    abstract：BACKGROUND:The molecular pathogenesis underlying recurrent exacerbations of atopic dermatitis (AD) is unclear. Some peripheral CCR4(+) and CCR7(+) helper memory T cells express the specific homing receptor, sialyl 6-sulfo Lewis X (G152 glycan). This glycan loses receptor activity via cyclization of its sialic acid moie...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/j.jdermsci.2012.09.015

    authors： Sakuma K,Furuhashi T,Kondo S,Yabe U,Ohmori K,Ito H,Aoki M,Morita A,Kannagi R

    更新日期：2012-12-01 00:00:00

  • Telomere length of the skin in association with chronological aging and photoaging.

    abstract：BACKGROUND:Telomere shortening has been implicated in cellular senescence, which may cause certain aging phenotypes. OBJECTIVE:To reveal whether telomere shortening is associated with chronological aging and/or photoaging of the skin, we measured telomere length in the epidermis and in the dermis from sun-protected an...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/j.jdermsci.2006.02.004

    authors： Sugimoto M,Yamashita R,Ueda M

    更新日期：2006-07-01 00:00:00

  • Spectrum of genetic variation at the ABCC6 locus in South Africans: Pseudoxanthoma elasticum patients and healthy individuals.

    abstract：BACKGROUND:Pseudoxanthoma elasticum (PXE) is an autosomal recessive metabolic disorder with ectopic mineralization in the skin, eyes and cardiovascular system. PXE is caused by mutations in ABCC6. OBJECTIVE:To examine 54 unrelated South African PXE patients for ABCC6 PXE causing mutations. METHODS:Patients were scree...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/j.jdermsci.2009.02.008

    authors： Ramsay M,Greenberg T,Lombard Z,Labrum R,Lubbe S,Aron S,Marais AS,Terry S,Bercovitch L,Viljoen D

    更新日期：2009-06-01 00:00:00

  • Crystalloid inclusion bodies of the endothelial cells in human fetal skin blood vessels and human umbilical cord vessels: a possible relationship to Weibel-Palade bodies?

    abstract：:Crystalloid inclusion bodies (CIB) of the endothelial cells (EC) were investigated in blood vessels of human fetal skin and the umbilical cord by electron- and immunoelectron microscopy. They were found in up to 15% of the investigated EC in various types of vessels. Their sizes ranged from 0.2 microns to 0.6 microns ...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/0923-1811(91)90020-x

    authors： Tronnier M,Hashimoto K,Setoyama M,Predeteanu GS

    更新日期：1991-03-01 00:00:00

  • Development of an inducible gene expression system for primary murine keratinocytes.

    abstract：BACKGROUND:The tetracycline (Tet) responsive system is a valuable tool that is routinely used in a wide variety of mammalian cells for regulatable expression of gene products. However, technical difficulties such as harsh selection conditions and extensive screening processes to identify suitably responsive clones limi...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/j.jdermsci.2007.09.003

    authors： Nagarajan P,Sinha S

    更新日期：2008-01-01 00:00:00

  • Vascular endothelial growth factor (VEGF) in the pathogenesis of psoriasis--a possible target for novel therapies?

    abstract：:Angiogenesis is defined as the formation of new capillaries from pre-existing blood vessels. The process of angiogenesis is tightly regulated by a balance between pro- and anti-angiogenic factors. Vascular endothelial growth factor (VEGF) is a pro-angiogenic factor and several anti-VEGF therapies are used in the treat...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章,评审

    doi：10.1016/j.jdermsci.2010.03.023

    authors： Canavese M,Altruda F,Ruzicka T,Schauber J

    更新日期：2010-06-01 00:00:00

  • Effect of cyclosporin A on suppressor T cells.

    abstract：:The effect of cyclosporin A (CsA) on suppressor T cells (Ts) was evaluated by in vitro incubation with afferent-phase Ts (Ts-aff) and efferent-phase Ts (Ts-eff). 2,4-Dinitro-1-fluorobenzene (DNFB) and hen egg-white lysozyme (HEL) were used as antigens. Both Ts-aff and Ts-eff were resistant to high- and low-dose CsA tr...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/0923-1811(90)90218-3

    authors： Ishii N,Ishii H,Ono H,Horiuchi Y,Nakajima H,Aoki I

    更新日期：1990-03-01 00:00:00

  • Characterization of itch-associated responses of NC mice with mite-induced chronic dermatitis.

    abstract：:To clarify the behavioral and pathological features of spontaneous scratching of NC mice with mite-induced chronic dermatitis, we investigated the spontaneous and pruritogen-evoked scratching of NC mice. Although the frequency of scratching of NC mouse did not increase under specific pathogen-free environment, it grad...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/s0923-1811(00)00099-2

    authors： Yamaguchi T,Maekawa T,Nishikawa Y,Nojima H,Kaneko M,Kawakita T,Miyamoto T,Kuraishi Y

    更新日期：2001-01-01 00:00:00

  • Tryptophan photo-product FICZ upregulates AHR/MEK/ERK-mediated MMP1 expression: Implications in anti-fibrotic phototherapy.

    abstract：BACKGROUND:Scleroderma is caused by aberrant transforming growth factor-ß signaling. The degradation of extracellular matrix proteins is regulated by matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs). Ultraviolet (UV) radiation has been a therapy for scleroderma. 6-Formylindolo[3,2-b]...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/j.jdermsci.2018.04.010

    authors： Murai M,Yamamura K,Hashimoto-Hachiya A,Tsuji G,Furue M,Mitoma C

    更新日期：2018-07-01 00:00:00

  • Differential regulations of matrix metalloproteinases and tissue inhibitors of metalloproteinases in dermal fibroblasts by dibutyril cyclic AMP.

    abstract：:The influence of dibutyril cyclic AMP (dbcAMP) on the gene expression of matrix metalloproteinases (MMP)-1, MMP-2, MMP-3, tissue inhibitors of metalloproteinases (TIMP)-1 and TIMP-2 was investigated in human skin fibroblasts by northern analyses. The treatment of dbcAMP increased MMP-1 and MMP-3 mRNA levels but decrea...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/s0923-1811(97)00035-2

    authors： Kuroda K,Shinkai H

    更新日期：1997-08-01 00:00:00

  • Ligand-activated PPARδ upregulates α-smooth muscle actin expression in human dermal fibroblasts: A potential role for PPARδ in wound healing.

    abstract：BACKGROUND:The phenotypic changes that accompany differentiation of resident fibroblasts into myofibroblasts are important aspects of the wound healing process. Recent studies showed that peroxisome proliferator-activated receptor (PPAR) δ plays a critical role in wound healing. OBJECTIVE:To determine whether the nucl...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/j.jdermsci.2015.10.005

    authors： Ham SA,Hwang JS,Yoo T,Lee WJ,Paek KS,Oh JW,Park CK,Kim JH,Do JT,Kim JH,Seo HG

    更新日期：2015-12-01 00:00:00

  • Inflammatory papillomatous hyperplasia and epidermal necrosis in a transgenic rat for HIV-1.

    abstract：BACKGROUND:Skin lesions commonly affect AIDS patients. The pathogenesis of certain dermatologic disorders primarily associated to HIV-1 is unclear, and better forms of therapy for these conditions need to be discovered. Transgenic animal models represent a novel approach for the study of these disorders and for the que...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/j.jdermsci.2008.08.015

    authors： Cedeno-Laurent F,Bryant J,Fishelevich R,Jones OD,Deng A,Eng ML,Gaspari AA,Trujillo JR

    更新日期：2009-02-01 00:00:00

  • Normal proliferative responses of peripheral blood mononuclear cells to streptococcal preparation OK-432 in patients with pustulosis palmaris et plantaris constitute a distinct feature from the reduced responses observed in those with psoriasis vulgaris,

    abstract：:It was previously reported that peripheral blood mononuclear cells (PBMC) from the patients with psoriasis vulgaris (PV) showed a reduced proliferative response in vitro to the stimulation of a lyophilized preparation of penicillin-treated low virulence Su-strain of Streptococcus pyogenes group 3, OK-432. In this stud...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/s0923-1811(00)00109-2

    authors： Takahashi K,Aiba S,Uddin Z,Kasai H,Tagami H

    更新日期：2001-01-01 00:00:00

  • Harlequin ichthyosis and other autosomal recessive congenital ichthyoses: the underlying genetic defects and pathomechanisms.

    abstract：:Autosomal recessive congenital ichthyoses (ARCI) include several severe subtypes including harlequin ichthyosis (HI), lamellar ichthyosis and non-bullous congenital ichthyosiform erythroderma. Patients with these severe types of ichthyoses frequently show severe hyperkeratosis and scales over a large part of the body ...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章,评审

    doi：10.1016/j.jdermsci.2006.01.003

    authors： Akiyama M

    更新日期：2006-05-01 00:00:00

  • Overactivation of IL-4-induced activator protein-1 in atopic dermatitis.

    abstract：:Atopic dermatitis is regarded as mediated by Th2-type immunity. In fact, it frequently coincides with the elevation of immunoglobulin (Ig)-E in patients' sera. Due to the pivotal role of interleukin (IL)-4 in regulation of IgE, we hypothesized if atopic dermatitis represents a hyper-reactive condition in response to I...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/s0923-1811(01)00171-2

    authors： Yamazaki F,Aragane Y,Maeda A,Matsushita K,Ueno K,Yudate T,Kawada A,Tezuka T

    更新日期：2002-04-01 00:00:00

  • Update on the mechanisms and efficacy of biological therapies for psoriasis.

    abstract：:Biologically based agents (biologics) are novel therapeutic options in the treatment of moderate-to-severe psoriasis. Unlike traditional systemic anti-psoriatic drugs, which are chemically synthesized, these agents are unique in that they are derived from living organisms and hence called "biologics." In addition, the...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章,评审

    doi：10.1016/j.jdermsci.2005.01.011

    authors： Koo J,Khera P

    更新日期：2005-05-01 00:00:00

  • Combined treatment with H1 and H4 receptor antagonists reduces inflammation in a mouse model of atopic dermatitis.

    abstract：BACKGROUND:Histamine 4 receptor (H4R) antagonists are considered as new therapeutics for the treatment of atopic dermatitis (AD) and first clinical trials have already shown promising results. Histamine 1 receptor (H1R) antagonists are traditionally used to treat AD although the evidence for the efficacy is weak. The c...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/j.jdermsci.2017.04.004

    authors： Köchling H,Schaper K,Wilzopolski J,Gutzmer R,Werfel T,Bäumer W,Kietzmann M,Rossbach K

    更新日期：2017-08-01 00:00:00

  • Neither cyclosporin A nor FK506 affects adenylate cyclase responses of fetal rat keratinizing epidermal cells (FRSK cells) at concentrations that inhibit thymidine incorporation.

    abstract：:It has been reported that various anti-psoriatic agents augment the beta-adrenergic adenylate cyclase response that is defective in the psoriatic hyperproliferative epidermis. Recent evidence indicates that cyclosporin A (CsA) and FK506 show beneficial effects on psoriasis. Using fetal rat keratinizing epidermal cells...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/0923-1811(93)90044-p

    authors： Takahashi H,Iizuka H

    更新日期：1993-12-01 00:00:00

  • Mechanism of action of androgen in hair follicles.

    abstract：:In order to investigate the mode of action of testosterone (T) on human hair follicles we studied the metabolism of T and localization of androgen receptors in outer root sheath cells (ORSC) and dermal papilla cells (DPC) from different body sites. T was principally metabolized to androstenedione (delta 4) even in bea...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/0923-1811(94)90040-x

    authors： Itami S,Sonoda T,Kurata S,Takayasu S

    更新日期：1994-07-01 00:00:00

  • Modulation of UVA light-induced oxidative stress by beta-carotene, lutein and astaxanthin in cultured fibroblasts.

    abstract：:The ability of beta-carotene, lutein or astaxanthin to protect against UVA-induced oxidative stress in rat kidney fibroblasts (NRK) was assessed. Activities of the antioxidant enzymes catalase (CAT) and superoxide dismutase (SOD), and changes in thiobarbituric acid reactive substances (TBARS) were measured as indices ...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/s0923-1811(97)00058-3

    authors： O'Connor I,O'Brien N

    更新日期：1998-03-01 00:00:00

  • rAAV2-mediated restoration of LEKTI in LEKTI-deficient cells from Netherton patients.

    abstract：BACKGROUND:Netherton syndrome (NS, MIM 256500) is a potential live threatening autosomal-recessive skin disorder clinically characterized by the trias of congenital erythroderma, hair shaft anomalies and atopic diathesis. It is caused by mutations in the gene SPINK5 resulting in a deficiency of its processed protein na...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/j.jdermsci.2010.12.004

    authors： Roedl D,Oji V,Buters JT,Behrendt H,Braun-Falco M

    更新日期：2011-03-01 00:00:00

  • Genetically engineered mouse models for skin research: taking the next step.

    abstract：:Genetically engineered mouse models are invaluable to investigators in nearly all areas of biomedical research. The use of genetically engineered mice has allowed researchers to explore fundamental functions of genes in a mammal that shares substantial similarities with human physiology and pathology. Genetically engi...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章,评审

    doi：10.1016/j.jdermsci.2008.03.012

    authors： Chen J,Roop DR

    更新日期：2008-10-01 00:00:00

  • Animal models for atopic dermatitis: are they relevant to human disease?

    abstract：:Over the last decade, animal models of atopic dermatitis (AD) have received increasing attention. They include NC/Nga mice, a hapten-induced mouse model, and transgenic and knockout mouse models. Although the pathogenesis of skin inflammation elicited in these models and that in AD are not quite the same, it is pertin...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章,评审

    doi：10.1016/j.jdermsci.2004.02.013

    authors： Shiohara T,Hayakawa J,Mizukawa Y

    更新日期：2004-10-01 00:00:00

  • Characterization of fibroblast-derived prolidase. The presence of two forms of prolidase.

    abstract：:Crude enzyme solutions of prolidase were extracted from cultured human skin fibroblasts derived from control and prolidase-deficient sisters. Two forms of prolidases (prolidase-I and II) were partially purified by high performance liquid chromatography equipped with an ion exchange column. On gel filtration, the relat...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/0923-1811(90)90588-5

    authors： Oono T,Yasutomi H,Ohhashi T,Kodama H,Arata J

    更新日期：1990-09-01 00:00:00

  • A novel niche for skin derived precursors in non-follicular skin.

    abstract：BACKGROUND:Skin derived precursors (SKP) comprise a subset of specialized dermal cells that can be distinguished from fibroblast by their capacity for spheroidal growth. Recent investigations have shown that hair follicles constitute a niche for this cell type, but their localization and their definite function in non-...

    journal_title：Journal of dermatological science

    pub_type： 杂志文章

    doi：10.1016/j.jdermsci.2012.10.007

    authors： Ruetze M,Knauer T,Gallinat S,Wenck H,Achterberg V,Maerz A,Deppert W,Knott A

    更新日期：2013-02-01 00:00:00