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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 2 forms of ichthyosis, autosomal recessive congenital ichthyosis and harlequin ichthyosis...
October 18, 2016: Journal of Investigative Dermatology
Jeffrey B Doyon, Kristina J Liu, Rebecca A Berman
No abstract text is available yet for this article.
October 19, 2016: Journal of General Internal Medicine
Annie Liu, Lyne Giroux
An 86-year-old man developed a suspected severe erythroderma during treatment with silodosin (dosage unknown) for benign prostatic enlargement. Two weeks after starting silodosin, he developed a total-body scaling dermatitis. A biopsy was planned but the patient improved at his subsequent visit and it was not taken. Silodosin was discontinued and the patient received UVB phototherapy, clobetasol ointment, and several bland and protective skin-care measures. One week after the initial presentation, the patient demonstrated improvement in his total-body scaling...
December 2015: Drug Saf Case Rep
A Paganelli, S Ciardo, G Odorici, G Pellacani, A Conti
Pityriasis rubra pilaris (PRP) is a rare, chronic inflammatory skin disorder characterized by the presence of small follicular hyperkeratotic papules that coalesce into yellow-pink (salmon-colored) patches, usually beginning at the head and neck and often resulting in generalized erythroderma(1) . Other main clinical features are "islands" of sparing within the affected skin, palmoplantar keratoderma, nail dystrophy, oral involvement and eventually ectropion. The age of onset has a peak between 50 and 70 years but a juvenile presentation is not uncommon...
October 14, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Rebecca Kohnken, Stephanie Fabbro, Justin Hastings, Pierluigi Porcu, Anjali Mishra
Sézary syndrome (SS) is a rare and aggressive type of cutaneous T cell lymphoma (CTCL) characterized by an intensely pruritic, exfoliative rash, known as erythroderma, with cutaneous and systemic dissemination of clonal CD4+ T cells into the blood and lymph nodes. This review aims to present recent advancements in the biological and clinical aspects of SS. We begin by providing an overview of the diagnostic criteria for SS and reviewing some of its epidemiological and clinical aspects. We then discuss updates in the etiology of this elusive disease and the genetic and molecular landscapes that define it...
October 4, 2016: Current Hematologic Malignancy Reports
Arpita Sen, Satyendranath Chowdhury, Indrasish Poddar, Debabrata Bandyopadhyay
INTRODUCTION: Dermatology is primarily a non-acute, outpatient-centered clinical specialty, but substantial number of patients need indoor admission for adequate management. Over the years, the need for inpatient facilities in Dermatology has grown manifold; however, these facilities are available only in some tertiary centers. AIMS AND OBJECTIVES: To analyze the characteristics of the diseases and outcomes of patients admitted in the dermatology inpatient Department of a tertiary care facility in eastern India...
September 2016: Indian Journal of Dermatology
Nadia Waheed, Huma Arshad Cheema, Hassan Suleman, Iqra Mushtaq, Zafar Fayyaz
Chanarin-Dorfman syndrome is a rare, genetically determined autosomal recessive disorder, characterised by the presence of lipid droplets in the cytoplasm of multiple tissues of the body, particularly in the blood leukocytes and congenital non-bullous icthyosiform erythroderma. In this paper, we report one-year child who presented with skin lesions since birth and hepatomegaly. Liver biopsy showed steatohepatitis; and peripheral blood smear confirmed Jordan`s anomaly, which is a permanent feature of Chanarin-Dorfman syndrome...
September 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
S Dănescu, J Leppert, R Cosgarea, S Zurac, S Pop, A Baican, C Has
SAM syndrome (OMIM 615508), comprising severe dermatitis, multiple allergies and metabolic wasting, was first described by Samuelov et al in 2013(1) . Cutaneous findings consisted of congenital erythroderma, superficial skin erosions, fine scales and palmoplantar keratoderma and were accompanied by extracutaneous findings including perinatal hyponatremia, food allergies, elevated IgE levels, recurrent infections, metabolic wasting, malabsorbtion, esophageal reflux and eosinophilic esophagitis, cardiac defects, microcephaly and development delay (2,3) ...
September 15, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Alina Jankowska-Konsur, Christopher Kobierzycki, Jedrzej Grzegrzolka, Aleksandra Piotrowska, Agnieszka Gomulkiewicz, Natalia Glatzel-Plucinska, Mateusz Olbromski, Marzenna Podhorska-Okolow, Jacek C Szepietowski, Piotr Dziegiel
BACKGROUND/AIM: Mycosis fungoides (MF) is the most common primary cutaneous T-cell lymphoma (CTCL) characterized by malignant proliferation of mature T lymphocytes and primary skin involvement. Recent reports suggest that angiogenesis may play a role in the growth and spread of this malignancy. Cluster of differentiation 31 (CD31) is a protein classified into the Ig-superfamily of cell adhesion molecules, expressed on endothelial cells and majority of hematopoietic non-erythroid cells...
September 2016: Anticancer Research
Enzo Errichetti, Giuseppe Stinco
Over the last few years, dermoscopy has been shown to be a useful tool in assisting the noninvasive diagnosis of various general dermatological disorders. In this article, we sought to provide an up-to-date practical overview on the use of dermoscopy in general dermatology by analysing the dermoscopic differential diagnosis of relatively common dermatological disorders grouped according to their clinical presentation, i.e. dermatoses presenting with erythematous-desquamative patches/plaques (plaque psoriasis, eczematous dermatitis, pityriasis rosea, mycosis fungoides and subacute cutaneous lupus erythematosus), papulosquamous/papulokeratotic dermatoses (lichen planus, pityriasis rosea, papulosquamous sarcoidosis, guttate psoriasis, pityriasis lichenoides chronica, classical pityriasis rubra pilaris, porokeratosis, lymphomatoid papulosis, papulosquamous chronic GVHD, parakeratosis variegata, Grover disease, Darier disease and BRAF-inhibitor-induced acantholytic dyskeratosis), facial inflammatory skin diseases (rosacea, seborrheic dermatitis, discoid lupus erythematosus, sarcoidosis, cutaneous leishmaniasis, lupus vulgaris, granuloma faciale and demodicidosis), acquired keratodermas (chronic hand eczema, palmar psoriasis, keratoderma due to mycosis fungoides, keratoderma resulting from pityriasis rubra pilaris, tinea manuum, palmar lichen planus and aquagenic palmar keratoderma), sclero-atrophic dermatoses (necrobiosis lipoidica, morphea and cutaneous lichen sclerosus), hypopigmented macular diseases (extragenital guttate lichen sclerosus, achromic pityriasis versicolor, guttate vitiligo, idiopathic guttate hypomelanosis, progressive macular hypomelanosis and postinflammatory hypopigmentations), hyperpigmented maculopapular diseases (pityriasis versicolor, lichen planus pigmentosus, Gougerot-Carteaud syndrome, Dowling-Degos disease, erythema ab igne, macular amyloidosis, lichen amyloidosus, friction melanosis, terra firma-forme dermatosis, urticaria pigmentosa and telangiectasia macularis eruptiva perstans), itchy papulonodular dermatoses (hypertrophic lichen planus, prurigo nodularis, nodular scabies and acquired perforating dermatosis), erythrodermas (due to psoriasis, atopic dermatitis, mycosis fungoides, pityriasis rubra pilaris and scabies), noninfectious balanitis (Zoon's plasma cell balanitis, psoriatic balanitis, seborrheic dermatitis and non-specific balanitis) and erythroplasia of Queyrat, inflammatory cicatricial alopecias (scalp discoid lupus erythematosus, lichen planopilaris, frontal fibrosing alopecia and folliculitis decalvans), nonscarring alopecias (alopecia areata, trichotillomania, androgenetic alopecia and telogen effluvium) and scaling disorders of the scalp (tinea capitis, scalp psoriasis, seborrheic dermatitis and pityriasis amiantacea)...
September 9, 2016: Dermatology and Therapy
Korbkarn Pongpairoj, Iris Ale, Klaus Ejner Andersen, Magnus Bruze, Thomas L Diepgen, Peter U Elsner, Chee Leok Goh, An Goossens, Hemangi Jerajani, Jean Marie Lachapelle, Jun Young Lee, Howard I Maibach, Kayoko Matsunaga, Rosemary Nixon, Pailin Puangpet, Denis Sasseville, Supitchaya Thaiwat, John P McFadden
The International Contact Dermatitis Research Group proposes a classification for the clinical presentation of contact allergy. The classification is based primarily on the mode of clinical presentation. The categories are direct exposure/contact dermatitis, mimicking or exacerbation of preexisting eczema, multifactorial dermatitis including allergic contact dermatitis, by proxy, mimicking angioedema, airborne contact dermatitis, photo-induced contact dermatitis, systemic contact dermatitis, noneczematous contact dermatitis, contact urticaria, protein contact dermatitis, respiratory/mucosal symptoms, oral contact dermatitis, erythroderma/exfoliative dermatitis, minor forms of presentation, and extracutaneous manifestations...
September 2016: Dermatitis
Brandon Rowe, Gil Yosipovitch
Paraneoplastic itch occurs as the result of a systemic reaction to an underlying malignancy. Paraneoplastic itch is most commonly associated with lymphoproliferative malignancies and solid tumors that result in cholestasis. Paraneoplastic itch may occur in the absence of a primary rash or in association with dermatologic conditions such as erythroderma, acanthosis nigricans, dermatomyositis, Grover's disease, and eruptive seborrheic keratosis. Treatment of paraneoplastic itch is centered on targeting the underlying malignancy responsible for the systemic reaction...
2016: Current Problems in Dermatology
Sarah L Greig
Brodalumab (Lumicef(®)) is a human monoclonal immunoglobulin G antibody that is being developed by Kyowa Hakko Kirin in Japan, where it has been approved for the treatment of psoriasis vulgaris, psoriatic arthritis, pustular psoriasis and psoriatic erythroderma. Brodalumab binds with high affinity to interleukin (IL)-17 receptor A, thereby inhibiting several pro-inflammatory cytokines from the IL-17 family. Regulatory applications for brodalumab in plaque psoriasis are also under review in the USA, EU and Canada...
September 2016: Drugs
M Ndiaye, M Taleb, B A Diatta, A Diop, M Diallo, S Diadie, N B Seck, S Diallo, M T Ndiaye, S O Niang, F Ly, A Kane, M T Dieng
INTRODUCTION: The etiologies of intertrigo in adults are numerous and different. The objective of our work was to study the epidemiological, clinical and the risk factors of intertrigo in adults. METHODS: We conducted a prospective study for a period of seven months in two Dermatology Units in Dakar (Senegal). All adults patient with intertrigo seen during this period who gave consent were included. RESULTS: One hundred and three patients with intertrigo were diagnosed with a hospital prevalence of 2...
August 20, 2016: Journal de Mycologie Médicale
Amy S Paller, Yael Renert-Yuval, Maria Suprun, Hitokazu Esaki, Margeaux Oliva, Thy Nhat Huynh, Benjamin Ungar, Norma Kunjravia, Rivka Friedland, Xiangyu Peng, Xiuzhong Zheng, Yeriel D Estrada, James G Krueger, Keith A Choate, Mayte Suárez-Fariñas, Emma Guttman-Yassky
BACKGROUND: The ichthyoses are rare genetic disorders associated with generalized scaling, erythema, and epidermal barrier impairment. Pathogenesis-based therapy is largely lacking, since the underlying molecular basis is poorly understood. OBJECTIVE: To characterize molecularly cutaneous inflammation and its correlation with clinical and barrier characteristics. METHODS: We analyzed biopsies from 21 genotyped ichthyosis patients (congenital ichthyosiform erythroderma (n=6), lamellar ichthyosis (n=7), epidermolytic ichthyosis, (n=5) and Netherton syndrome (n=3)) by immunohistochemistry and RT-PCR and compared them with healthy controls, and atopic dermatitis (AD) and psoriasis patients...
August 20, 2016: Journal of Allergy and Clinical Immunology
Sean Whittaker
van Doorn et al. have defined the DNA methylomes of Sézary cells based on a genome-wide methylation analysis using the Illumina 450K array platform (Illumina, San Diego, CA). Their results show aberrant DNA methylation patterns in CD4-enriched T cells from peripheral blood samples, patterns that are distinct from those of patients with inflammatory erythroderma and from healthy volunteers. Whereas 7.8% of 473,921 5'-cytosine-phosphate-guanine-3' (CpG) sites were hypomethylated, 3.2% showed marked enrichment and selection for hypermethylated CpG sites within the proximal region of gene promoters, including some genes that have previously been shown to be hypermethylated in cutaneous T-cell lymphomas (CTCLs), using standard bisulfite modification techniques...
September 2016: Journal of Investigative Dermatology
D Miyamoto, M N Sotto, C S V Otani, L M I Fukumori, N V Pereira, C G Santi, C W Maruta, M N N Burnier, M M Rebeis, V Aoki
BACKGROUND: Erythroderma is a clinical skin syndrome shared by patients with cutaneous disorders of distinct etiologies as a result of the combined actions of chemokines, adhesion molecules, and cytokines, such as vascular endothelial growth factor (VEGF). OBJECTIVE: To evaluate the profile of serum levels of VEGF and soluble vascular endothelial growth factor receptor 1 (sVEGFR-1) in pemphigus foliaceus (PF) patients with erythroderma. METHODS: We conducted a retrospective study, which included 1) a chart review of all PF patients from the Autoimmune Blistering Clinic, University of Sao Paulo, Brazil, from January 1991 to December 2014, together with an evaluation of demographic variables, hospitalization duration and complications and 2) analysis of the circulating VEGF and sVEGFR-1 levels in PF patients with erythroderma by ELISA...
August 11, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Travis Tagami, Omar Alhalabi, Nicholas Ward, James Huang
BACKGROUND/AIMS: Paraneoplastic dermatoses are skin disorders that are associated with malignancy. Anaplastic large T-cell lymphoma (ALTCL) has rarely been associated with paraneoplastic skin manifestations such as gangrenous foot ulcers and erythroderma. METHODS: We describe a case of ALTCL presenting as a large annular skin rash. The clinical picture, course, and treatment will be discussed along with current hypotheses on the mechanism of paraneoplastic syndromes...
April 2016: Dermatopathology (Basel, Switzerland)
Takayoshi Komatsu-Fujii, Sakae Kaneko, Yuko Chinuki, Yohji Suyama, Masataka Ohta, Hiroyuki Niihara, Eishin Morita
BACKGROUND: This study aims to evaluate the relationship between serum thymus and activation-regulated chemokine (TARC) levels with various clinicopathological conditions in patients with drug eruptions. The value of TARC in diagnosing drug-induced hypersensitivity syndrome (DIHS) was also examined. METHODS: Study participants included 84 patients who presented with generalized eruptions suspected to be drug-related, including DIHS, Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN), maculopapular exanthema (MPE), erythema multiforme (EM), erythroderma, and toxicoderma...
August 4, 2016: Allergology International: Official Journal of the Japanese Society of Allergology
R Gruber, G Rainer, A Weiss, A Udvardi, H Thiele, K M Eckl, R Schupart, P Nürnberg, J Zschocke, M Schmuth, B Volc-Platzer, H C Hennies
Autosomal recessive congenital ichthyosis (ARCI) caused by mutations in CYP4F22 is very rare. CyP4F22, a protein of the cytochrome-P450 family 4, encodes an epidermal ω-hydroxylase decisive for the formation of acylceramides, which is hypothesised to be crucial for skin barrier function. We report a girl with consanguineous parents presenting as collodion baby with contractures of the great joints and palmoplantar hyperlinearity. In the course of the disease she developed fine scaling of the skin with erythroderma, the latter disappearing until the age of six months...
July 23, 2016: British Journal of Dermatology
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