The Experts below are selected from a list of 1488 Experts worldwide ranked by ideXlab platform
Adrian Tanew - One of the best experts on this subject based on the ideXlab platform.
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successful use of acitretin in conjunction with narrowband ultraviolet b phototherapy in a child with severe Pustular Psoriasis von zumbusch type
British Journal of Dermatology, 2004Co-Authors: T Kopp, Franz Karlhofer, Zsolt Szepfalusi, A Schneeberger, Georg Stingl, Adrian TanewAbstract:Summary Severe Pustular Psoriasis von Zumbusch type is a therapeutic challenge not only in adults, but even more in children. We report a 3½-year-old boy who developed a generalized flare of diffusely scattered pustules on erythematous skin which rapidly progressed to large exuding areas. The clinical presentation and investigations including histopathological examination of a biopsy and negative bacterial cultures were consistent with the diagnosis of Pustular Psoriasis von Zumbusch type. Upon initial treatment with methylprednisolone, acitretin and antibiotics the extent of the disease declined. However, several attempts to reduce the dose of the oral corticosteroid were followed by immediate severe flares. Additional treatment with narrowband ultraviolet B (NB-UVB, 311–313 nm UVB) resulted in a rapid arrest of disease activity and allowed the corticosteroid to be tapered off. After 10 irradiations the patient was both off steroid and disease free. NB-UVB therapy was subsequently reduced to twice-weekly exposures and acitretin gradually diminished to a maintenance dose of 0·3 mg kg−1 daily. We conclude that NB-UVB in conjunction with acitretin is a potent therapeutic regimen for the treatment of severe Pustular Psoriasis von Zumbusch type in childhood.
Herve Bachelez - One of the best experts on this subject based on the ideXlab platform.
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Pustular Psoriasis and related Pustular skin diseases.
The British journal of dermatology, 2018Co-Authors: Herve BachelezAbstract: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. These insights have unveiled pathophysiological features that shift the existing paradigms and emphasize the autoinflammatory nature of skin Pustular disorders. They also highlight the crucial role of the innate immune system across entities belonging to the Psoriasis disease spectrum, allowing identification of new appealing therapeutic targets.
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partial clinical response to anakinra in severe palmoplantar Pustular Psoriasis
British Journal of Dermatology, 2014Co-Authors: Manuelle Viguier, Herve Bachelez, M Tauber, E Alimova, A Petit, F Liote, A SmahiAbstract:Summary Background Palmoplantar Pustular Psoriasis is a clinical Psoriasis variant characterised by a high impact on quality of life and poor response to biologics approved for plaque type Psoriasis.The recombinant interleukin-1 (IL-1) receptor antagonist anakinra has been recently used for the treatment of isolated refractory cases of generalised Pustular Psoriasis with contrasted results. Objectives To report the clinical response in two patients treated with anakinra as salvage therapy in two patients with severe palmoplantar Pustular Psoriasis refractory to currently available antipsoriatic systemic therapies. Methods Anakinra was given subcutaneously at the daily dose of 100 mg, and clinical response was evaluated using the palmoplantar Psoriasis area and severity index (PPPASI). Results Only partial and transient responses were observed in both patients, who had to stop anakinra due to lack of efficacy and to side effects. Conclusion Anakinra appears to provide only partial clinical improvement in refractory palmoplantar Pustular Psoriasis. Prospective clinical studies on larger populations are warranted to investigate more accurately both efficacy and safety of IL-1-inhibiting strategies in Pustular Psoriasis.
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ap1s3 mutations are associated with Pustular Psoriasis and impaired toll like receptor 3 trafficking
American Journal of Human Genetics, 2014Co-Authors: Niovi Settakaffetzi, Huichun Lu, Michael Duckworth, Michael H. Allen, Alexander A Navarini, Michael A Simpson, V. Patel, Herve Bachelez, David A BurdenAbstract:Adaptor protein complex 1 (AP-1) is an evolutionary conserved heterotetramer that promotes vesicular trafficking between the trans-Golgi network and the endosomes. The knockout of most murine AP-1 complex subunits is embryonically lethal, so the identification of human disease-associated alleles has the unique potential to deliver insights into gene function. Here, we report two founder mutations (c.11T>G [p.Phe4Cys] and c.97C>T [p.Arg33Trp]) in AP1S3, the gene encoding AP-1 complex subunit σ1C, in 15 unrelated individuals with a severe autoinflammatory skin disorder known as Pustular Psoriasis. Because the variants are predicted to destabilize the 3D structure of the AP-1 complex, we generated AP1S3-knockdown cell lines to investigate the consequences of AP-1 deficiency in skin keratinocytes. We found that AP1S3 silencing disrupted the endosomal translocation of the innate pattern-recognition receptor TLR-3 (Toll-like receptor 3) and resulted in a marked inhibition of downstream signaling. These findings identify Pustular Psoriasis as an autoinflammatory phenotype caused by defects in vesicular trafficking and demonstrate a requirement of AP-1 for Toll-like receptor homeostasis.
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high frequency of cholestasis in generalized Pustular Psoriasis evidence for neutrophilic involvement of the biliary tract
Hepatology, 2004Co-Authors: Manuelle Viguier, Marc Lemann, Anne-marie Zagdanski, Philippe Bertheau, Eric De Kerviler, Matthieu Allez, Michel Rybojad, Louis Dubertret, P. Morel, Herve BachelezAbstract:Generalized Pustular Psoriasis is a rare form of Psoriasis that is sometimes associated with extracutaneous manifestations. Evidence for biliary involvement has been suggested in isolated cases. We investigated the prevalence and nature of liver abnormalities occurring in this disease. Twenty-two patients consecutively admitted for generalized Pustular Psoriasis who underwent liver biological tests at the time of the attack and during the following weeks were included. Twenty patients (90%) had at least one abnormal biological liver parameter. Eleven patients (50%) had pronounced abnormalities: jaundice (4/22), gammaglutamyl transferase higher than 5 times the normal value (10/22), alkaline phosphatase higher than twice the normal value (7/22), and/or aminotransferases higher than 3 times the normal value (7/22). These abnormalities returned to normal range at the time of remission of Pustular Psoriasis, suggesting that severe liver abnormalities could be associated with severe cutaneous disease. Neutrophilic cholangitis was observed on liver biopsy. Persistent magnetic resonance cholangiopancreatography features similar to those observed in sclerosing cholangitis were found in 3 of the 4 patients studied. No causal factor other than Pustular Psoriasis could be identified. In conclusion, our results demonstrate the high frequency of liver abnormalities in patients with generalized Pustular Psoriasis. Biliary involvement related to neutrophilic cholangitis should be added to the spectrum of extracutaneous manifestations of this disease, and physicians should be aware of such a complication. (HEPATOLOGY 2004;40:452–458.)
Keiji Iwatsuki - One of the best experts on this subject based on the ideXlab platform.
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long term efficacy and safety of ixekizumab in japanese patients with erythrodermic or generalized Pustular Psoriasis subgroup analyses of an open label phase 3 study uncover j
Journal of The European Academy of Dermatology and Venereology, 2019Co-Authors: Yukari Okubo, Hoda Elmaraghy, Yoji Morisaki, Keiji Iwatsuki, Tomotaka Mabuchi, H Torisuitakura, Ko NakajoAbstract:Background Erythrodermic and generalized Pustular Psoriasis are rare, difficult to treat forms of Psoriasis. In previous reports, we documented 24- and 52-week findings of an open-label, phase 3 trial (UNCOVER-J) of ixekizumab in Japanese patients with erythrodermic or generalized Pustular Psoriasis; most patients responded to treatment and maintained response through 52 weeks. Objective To assess the long-term (>3 years) efficacy and safety of ixekizumab in Japanese patients with erythrodermic or generalized Pustular Psoriasis. Methods These subgroup analyses were of a partial population of patients from UNCOVER-J (NCT01624233; Sponsored by Eli Lilly and Company), specifically those with erythrodermic Psoriasis (N = 8) or generalized Pustular Psoriasis (N = 5). These patients received 160 mg ixekizumab at Week 0, ixekizumab 80 mg every 2 weeks through Week 12, and ixekizumab 80 mg every 4 weeks thereafter up to Week 244. This regimen is consistent with the regimen approved in Japan for plaque, erythrodermic, and generalized Pustular Psoriasis and psoriatic arthritis. Efficacy assessments included Global Improvement Score (GIS), Psoriasis Area and Severity Index (PASI), dermal symptoms (for patients with generalized Pustular Psoriasis), Dermatology Life Quality Index (DLQI) and Itch Numeric Rating Scale (NRS). Safety assessments included treatment-emergent adverse events and adverse events of special interest. Results Most patients had a GIS of resolved or improved from Week 12 onwards, and all patients had early and sustained improvement in PASI and dermal symptom (generalized Pustular Psoriasis only) scores. Mean improvements in DLQI and Itch NRS at Week 12 were sustained through Week 244. Ixekizumab was well tolerated over 3 years of treatment in patients with erythrodermic Psoriasis or generalized Pustular Psoriasis, and no new safety concerns were identified. Conclusion These findings suggest that ixekizumab can be an effective long-term treatment option for erythrodermic or generalized Pustular Psoriasis.
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annular Pustular Psoriasis with a heterozygous il36rn mutation
European Journal of Dermatology, 2015Co-Authors: Tomoko Miyake, Hiroshi Umemura, Hiroko Doi, Junko Kousogabe, Kazuhide Tsuji, Toshihisa Hamada, Kazumitsu Sugiura, Yumi Aoyama, Masashi Akiyama, Keiji IwatsukiAbstract:Annular Pustular Psoriasis (APP) is a rare form of Pustular Psoriasis, characterized by erythematous, annular or polycyclic lesions, eruptions of small sterile pustules and fine desquamation without systemic inflammation [1]. APP is a chronic and recurrent disease but has a benign course, contrasting with generalized Pustular Psoriasis (GPP), which is another severe form of Pustular Psoriasis with systemic inflammatory response syndrome [2]. Recent studies in Japan have shown that the majority of [...]
Nikhil Yawalkar - One of the best experts on this subject based on the ideXlab platform.
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Innate immune cells express IL-17A/F in acute generalized exanthematous pustulosis and generalized Pustular Psoriasis
Archives of Dermatological Research, 2014Co-Authors: Masato Kakeda, Christoph Schlapbach, Gabriela Danelon, M M Tang, Valentina Cecchinato, Nikhil Yawalkar, Mariagrazia UguccioniAbstract:Acute generalized exanthematous pustulosis (AGEP) and generalized Pustular Psoriasis (GPP) are rare Pustular skin disorders with systemic involvement. IL-17A/F is a proinflammatory cytokine involved in various neutrophilic inflammatory disorders. Here we show that IL-17A/F is highly expressed by innate immune cells such as neutrophils and mast cells in both AGEP and GPP.
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innate immune cells express il 17a f in acute generalized exanthematous pustulosis and generalized Pustular Psoriasis
Archives of Dermatological Research, 2014Co-Authors: Masato Kakeda, Christoph Schlapbach, Gabriela Danelon, M M Tang, Valentina Cecchinato, Nikhil Yawalkar, Mariagrazia UguccioniAbstract:Acute generalized exanthematous pustulosis (AGEP) and generalized Pustular Psoriasis (GPP) are rare Pustular skin disorders with systemic involvement. IL-17A/F is a proinflammatory cytokine involved in various neutrophilic inflammatory disorders. Here we show that IL-17A/F is highly expressed by innate immune cells such as neutrophils and mast cells in both AGEP and GPP.
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Alitretinoin abrogates innate inflammation in palmoplantar Pustular Psoriasis
British Journal of Dermatology, 2012Co-Authors: N. Irla, Alexander A Navarini, Nikhil YawalkarAbstract:Background Palmoplantar Pustular Psoriasis is often recalcitrant to therapy. Here we evaluated the therapeutic effect of alitretinoin in patients with recalcitrant palmoplantar Pustular Psoriasis and investigated subsequent immunopathological alterations. Methods Seven patients with palmoplantar Pustular Psoriasis were treated with oral alitretinoin 30 mg once daily for 12 weeks. Efficacy was assessed by palmoplantar Pustular Psoriasis area and severity index (PPPASI), visual analogue scales (VAS) on intensity of pain and pruritus and an overall patient assessment. Immunohistochemical staining for neutrophil elastase, CD3, CD4, CD8, CD1a CD11c, CD303,CD68, CD69, CD208 and HLA-DR was on lesional skin biopsies obtained before and after 12 weeks of treatment. Results PPPASI and VAS for pruritus and pain decreased significantly after 12 weeks of treatment with alitretinoin. The overall patient assessment ranged from 60% to 90% clinical improvement. In correlation with clinical improvement a significant reduction, particularly of neutrophils, macrophages and dendritic cells, was also observed in the skin sections. Alitretinoin was well tolerated except for headache during the first month of treatment in two patients. Limitations of the study are a missing control group and the concomitant usage of topical therapy. Discussion Our findings suggest that alitretinoin may represent a new and promising therapy for recalcitrant palmo-plantar Psoriasis and warrants further controlled studies to confirm efficacy and safety of alitretinoin in this disease.
Stan Pavel - One of the best experts on this subject based on the ideXlab platform.
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acitretin induces capillary leak syndrome in a patient with Pustular Psoriasis
Journal of The American Academy of Dermatology, 2007Co-Authors: Lydia E Vos, Maarten H Vermeer, Stan PavelAbstract:Capillary leak syndrome is a rare and potentially life-threatening condition caused by a shift of intravascular fluid and proteins to the interstitial space. We describe a patient with Pustular Psoriasis in whom capillary leak syndrome developed after the start of acitretin. Immediate withdrawal of retinoic acid is necessary and corticosteroid therapy should be considered.
