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Erythroderma

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https://www.readbyqxmd.com/read/28073814/donor-derived-mycosis-fungoides-following-reduced-intensity-haematopoietic-stem-cell-transplantation-from-a-matched-unrelated-donor
#1
Francesca A M Kinsella, Mohammad Rasoul Amel Kashipaz, Julia Scarisbrick, Ram Malladi
A 46-year-old woman with a history of dasatinib-resistant chronic myeloid leukaemia, clonal evolution and monosomy 7 underwent reduced intensity conditioned in vivo T-cell-depleted allogeneic haematopoietic stem cell transplantation (HSCT) from a matched unrelated donor. Following the transplantation, she developed recurrent cutaneous graft versus host disease (GvHD), which required treatment with systemic immunosuppression and electrocorporeal photophoresis. Concurrently, she developed a lichenoid rash with granulomatous features suggestive of cutaneous sarcoidosis...
January 10, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28058540/juvenile-dermatomyositis-a-tertiary-center-experience
#2
Kenan Barut, Pinar Ozge Avar Aydin, Amra Adrovic, Sezgin Sahin, Ozgur Kasapcopur
Juvenile dermatomyositis (JDM) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common idiopathic inflammatory myopathy of childhood. This study aimed to evaluate demographic and clinical features, laboratory data, treatment modalities, and outcome of patients with JDM at a referral pediatric rheumatology center in Turkey. We retrospectively reviewed medical records of patients diagnosed with JDM between the years 2003-2016 at the Pediatric Rheumatology Department Cerrahpasa Medical Faculty...
January 5, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28012574/s%C3%A3-zary-syndrome-without-erythroderma-a-review-of-16-cases-at-mayo-clinic
#3
Agnieszka K Thompson, Jill M Killian, Amy L Weaver, Mark R Pittelkow, Mark D P Davis
BACKGROUND: Sézary syndrome (SS) is characterized by erythroderma with leukemic involvement. In atypical SS, leukemic involvement is present without erythroderma. Little is known about the presentation, prognosis, and outcome in these patients. OBJECTIVE: We sought to describe our experience with patients with SS without erythroderma. METHODS: We retrospectively identified patients with SS, but without erythroderma, at Mayo Clinic from 1976 to 2010...
December 21, 2016: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/27958625/genital-psoriasis-a-hidden-multidisciplinary-problem-a-review-of-literature
#4
Piotr Czuczwar, Anna Stępniak, Andy Goren, Wojciech Wrona, Tomasz Paszkowski, Mariola Pawlaczyk, Dorota Piekarska-Myślińska, Sławomir Woźniak, Aldona Pietrzak
Genital psoriasis is a variety of autoimmune dermatological disease - psoriasis with relapsing-remitting course, which can have an onset in all age groups. It is most often diagnosed at an advanced stage. Genital psoriasis is considered an embar-rassing condition and is often misjudged as a sexually transmitted disease or allergic reaction due to low social awareness of the disease. The manifestations of genital psoriasis may differ from typical genital dermatoses and with symptoms such as itch, erythroderma and vaginal discharge may mimic other diseases at an early stage...
2016: Ginekologia Polska
https://www.readbyqxmd.com/read/27942369/erythrodermic-psoriasis-treated-with-apremilast
#5
John Arcilla, Daniel Joe, Johnathan Kim, Yohanan Kim, VuAnh N Truong, Navin Jaipaul
Erythroderma is a rare potentially deadly exfoliative dermatitis characterized by diffuse cutaneous erythema which may be associated with multi-organ dysfunction. Therefore, it is imperative to recognize and treat it promptly. Erythrodermic psoriasis is the most common form of erythroderma. Management of this condition is largely based on aggressive supportive care and the use of anti-inflammatory immunosuppressive and biologic agents. We describe a case of psoriatic erythroderma which was triggered by withdrawal from systemic steroids and successfully treated with apremilast and cyclosporine...
June 15, 2016: Dermatology Reports
https://www.readbyqxmd.com/read/27920816/concomitant-extraspinal-hyperostosis-and-osteoporosis-in-a-patient-with-congenital-ichthyosis
#6
Niloufar Torkamani, Pramit Phal, Ravi Savarirayan, Peter Simm, George Varigos, John Wark
Ichthyosiform dermatosis is a term referred to a group of disorders that have as their basis a disorder of keratinization (1). These conditions which are present at birth result in a generalized dry, scaly skin without any inflammation. There are several types of ichthyosis based on their clinical presentation and mode of inheritance. The most common types are: ichthyosis vulgaris, X-linked recessive ichthyosis, epidermolytic hyperkeratosis (bullous), lamellar ichthyosis and non-bullous ichthyosiform erythroderma...
May 2016: Clinical Cases in Mineral and Bone Metabolism
https://www.readbyqxmd.com/read/27904177/a-clinicopathological-analysis-of-primary-cutaneous-lymphomas-a-6-year-observational-study-at-a-tertiary-care-center-of-south-india
#7
Anza Khader, Shiny Padinjarayil Manakkad, Mohammed Shaan, Sarita Sasidharan Pillai, Najeeba Riyaz, P Binitha Manikoth, Muhammed Kunnummel, Sunitha Balakrishnan
BACKGROUND: Little data are available concerning clinical and pathological patterns of cutaneous lymphomas in India. AIM: To analyze the clinical and histopathological characteristics of cutaneous lymphomas in Indian patients. MATERIALS AND METHODS: This is a single-center, prospective, observational study carried out from January 1, 2010, to December 31, 2015. The patients underwent clinical examination, human T-cell lymphotropic virus-1 (HTLV-1) screening, skin biopsy with hematoxylin and eosin and immunohistochemistry staining...
November 2016: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/27882586/efficacy-and-safety-of-dose-escalation-of-infliximab-therapy-in-japanese-patients-with-psoriasis-results-of-the-spread-study
#8
Hideshi Torii, Masayuki Nakano, Toshiro Yano, Kazuoki Kondo, Hidemi Nakagawa
Although infliximab is approved for psoriasis, its efficacy is reduced over time in some patients. The aim of this phase III trial is to evaluate efficacy and safety of infliximab dose escalation in Japanese psoriasis patients with loss of efficacy to standard-dose therapy. Patients with plaque psoriasis, psoriatic arthritis, pustular psoriasis or psoriatic erythroderma who showed loss of efficacy to standard-dose therapy received infliximab dose escalation (10 mg/kg every 8 weeks) from weeks 0 to 32. Loss of efficacy was defined as not maintaining 50% reduction in the Psoriasis Area and Severity Index (PASI 50) after achieving PASI 75...
November 24, 2016: Journal of Dermatology
https://www.readbyqxmd.com/read/27873737/erythroderma-and-non-hodgkin-t-cell-lymphoma-what-else-apart-from-mycosis-fungoides-and-s%C3%A3-zary-syndrome
#9
Alessandro Pileri, Cinzia Pellegrini, Claudio Agostinelli, Vieri Grandi, Annalisa Patrizi, Pier Luigi Zinzani, Nicola Pimpinelli
BACKGROUND: Peripheral T-cell lymphomas, not otherwise specified (PTCL-NOS), are a rare condition characterised by specific histology, nodal presentation, and a poor prognosis. In total, 10-18% of patients present with cutaneous involvement which is regarded as a poor prognostic marker. However, cutaneous PTCL-NOS lesions have been rarely reported in the literature. OBJECTIVES: We sought to describe PTCL-NOS cases characterised by erythrodermic dissemination to the skin...
November 21, 2016: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/27868142/a-japanese-case-of-ichthyosiform-erythroderma-with-a-novel-mutation-in-of-nipal4-ichthyin
#10
Minori Kusakabe, Makoto Nagai, Eiji Nakano, Orie Jitsukawa, Chikako Nishigori, Kiyofumi Yamanishi
is missing (Short communication).
October 14, 2016: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/27862277/omenn-syndrome-presenting-with-striking-erythroderma-and-extreme-lymphocytosis-in-a-newborn
#11
Rabia Zafar, Aaron Ver Heul, Avraham Beigelman, Jeffrey J Bednarski, Susan J Bayliss, Louis P Dehner, Ilana S Rosman, Carrie C Coughlin
Omenn syndrome is an autosomal recessive form of "leaky" severe combined immune deficiency resulting in distinct phenotypic features. The patient described herein had an atypical presentation of Omenn syndrome, with conspicuous erythroderma and extreme lymphocytosis at birth, in contrast to the typical evolution of rash seen during the first few weeks of life. In addition, the skin findings were secondary to infiltration of CD8(+) (cytotoxic) T-cells in contrast to the CD4(+) (helper) T-cells typically seen, which broadens the Omenn syndrome phenotype...
November 12, 2016: Pediatric Dermatology
https://www.readbyqxmd.com/read/27846018/clearance-of-erythroderma-in-a-patient-on-apremilast-and-positive-patch-test-reactions-while-on-treatment
#12
Cameron E West, Joseph F Fowler
No abstract text is available yet for this article.
November 2016: Dermatitis
https://www.readbyqxmd.com/read/27837048/cutaneous-improvement-in-refractory-adult-and-juvenile-dermatomyositis-after-treatment-with-rituximab
#13
Rohit Aggarwal, Priyadarshini Loganathan, Diane Koontz, Zengbiao Qi, Ann M Reed, Chester V Oddis
OBJECTIVE: The aim was to assess the efficacy of rituximab for the cutaneous manifestations of adult DM and JDM. METHODS: Patients with refractory adult DM (n = 72) and JDM (n = 48) were treated with rituximab in a randomized placebo-phase-controlled trial [either rituximab early drug (week 0/1) or rituximab late arms (week 8/9), such that all subjects received study drug]. Stable concomitant therapy was allowed. Cutaneous disease activity was assessed using the Myositis Disease Activity Assessment Tool, which grades cutaneous disease activity on a visual analog scale...
November 11, 2016: Rheumatology
https://www.readbyqxmd.com/read/27828640/alemtuzumab-in-refractory-s%C3%A3-zary-syndrome
#14
Carmen María Alcántara Reifs, Rafael Salido-Vallejo, Gloria María Garnacho-Saucedo, Sofía De la Corte-Sánchez, Alberto González-Menchen, Antonio Vélez García-Nieto
Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by the triad of erythroderma, lymphadenopathy and circulating atypical cells. The emergence of new molecular targets has enabled the development of drugs such as alemtuzumab, an anti-CD52 monoclonal antibody, which has shown promising results in the treatment of this entity. We report the case of a 70-year-old male with refractory Sézary syndrome in whom treatment with alemtuzumab achieved an 80% skin lesion clearance with complete haematologic and radiologic response...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/27813222/invasive-melanoma-in-a-patient-with-congenital-ichthyosiform-erythroderma
#15
Prajakta Jaju, Roberto A Novoa, Susan M Swetter, Kavita Y Sarin
We describe the case of a 26-year-old woman with a history of congenital ichthyosiform erythroderma (CIE) who initially presented with a stage IIA amelanotic melanoma on her forearm that was surgically excised. We also review the literature on CIE-associated skin cancers and discuss the possible contribution of ichthyosis to the risk of cutaneous malignancies. Our findings emphasize the importance of close lifelong skin cancer screening in individuals with CIE and highlight the unique malignancy risk of these individuals...
November 4, 2016: Pediatric Dermatology
https://www.readbyqxmd.com/read/27792043/the-role-of-18f-fdg-pet-and-pet-ct-in-the-evaluation-of-primary-cutaneous-lymphoma
#16
Lin Qiu, Guojian Tu, Jing Li, Yue Chen
Primary cutaneous lymphoma (PCL) is the second most common type of extranodal non-Hodgkin lymphoma, including both cutaneous T-cell and B-cell lymphomas. PCL comprises numerous subtypes and thus has myriad clinical presentations in the skin and subcutaneous tissues. Accurate classification and staging are important for making treatment recommendations for PCL and will further impact patient prognosis significantly. We review the role of fluorine-18-fluorodeoxyglucose (F-FDG) PET (F-FDG PET) and F-FDG PET with computed tomography (CT) in the diagnosis, staging, tumor biological evaluation, treatment response assessment, and early recurrence surveillance of PCL...
October 27, 2016: Nuclear Medicine Communications
https://www.readbyqxmd.com/read/27790691/image-gallery-erythroderma-revealing-a-nonbullous-bullous-pemphigoid
#17
F Huet, A Karam, G Lemasson, F Jouen, H Sonbol, L Misery, C Abasq-Thomas
No abstract text is available yet for this article.
November 2016: British Journal of Dermatology
https://www.readbyqxmd.com/read/27771982/systemic-cyclosporine-treatment-in-severe-childhood-psoriasis-a-retrospective-chart-review
#18
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/27771215/zinc-deficiency-presenting-as-diarrhea-and-diffuse-erythroderma
#19
Rachel Aviv, Jennifer Toh, Rachel Eisenberg, Sarika Khanna, Sunit P Jariwala
No abstract text is available yet for this article.
December 2016: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/27769845/calpain-12-function-revealed-through-the-study-of-an-atypical-case-of-autosomal-recessive-congenital-ichthyosis
#20
Ron Bochner, Liat Samuelov, Ofer Sarig, Qiaoli Li, Christopher A Adase, Ofer Isakov, Natalia Malchin, Dan Vodo, Ronna Shayevitch, Alon Peled, Benjamin D Yu, Gilad Fainberg, Emily Warshauer, Noam Adir, Noam Erez, Andrea Gat, Yehonatan Gottlieb, Tova Rogers, Mor Pavlovsky, Ilan Goldberg, Noam Shomron, Aileen Sandilands, Linda E Campbell, Stephanie MacCallum, W H Irwin McLean, Gil Ast, Richard L Gallo, Jouni Uitto, Eli Sprecher
Congenital erythroderma is a rare and often life-threatening condition, which has been shown to result from mutations in several genes encoding important components of the epidermal differentiation program. Using whole exome sequencing, we identified in a child with congenital exfoliative erythroderma, hypotrichosis, severe nail dystrophy and failure to thrive, two heterozygous mutations in ABCA12 (c.2956C>T, p.R986W; c.5778+2T>C, p. G1900Mfs*16), a gene known to be associated with two forms of ichthyosis, autosomal recessive congenital ichthyosis, and harlequin ichthyosis...
October 18, 2016: Journal of Investigative Dermatology
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