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Skin fragility

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https://www.readbyqxmd.com/read/29457018/nail-changes-in-early-mycosis-fungoides
#1
Amir Hooshang Ehsani, Maryam Nasimi, Arghavan Azizpour, Pedram Noormohammadpoor, Kambiz Kamyab, Hasan Seirafi, Mojghan Noori
Background: Mycosis fungoides (MF) has a wide range of clinical presentations and it has been reported rarely to involve the nail apparatus. Objective: We intended to evaluate the frequency and characteristics of nail changes in patients with biopsy-proven MF. Methods: A retrospective analysis of 60 patients with MF who were evaluated at our cancer center from 2013 to 2014 was performed to identify patients with nail changes. Histological examinations of the skin around the nail apparatus were obtained from 10 patients with periungual skin erythema and scaling...
January 2018: Skin Appendage Disorders
https://www.readbyqxmd.com/read/29413767/the-role-of-intramedullary-fixation-in-ankle-fractures-a-systematic-review
#2
REVIEW
R W Jordan, A W P Chapman, D Buchanan, P Makrides
BACKGROUND: Ankle fractures are one of the most commonly occurring fractures in the elderly population. The overall incidence has been reported to be up to 184 fractures per 100,000 persons per year, of which 20-30% occur in the elderly. Medical co-morbidities, osteoporosis, suboptimal skin quality and poor toleration of non-weight bearing status all contribute to difficulties in managing these injuries in this population. Intramedullary implants are advantageous as they utilise smaller incisions, minimise soft tissue disruption and may allow early weight bearing...
February 2018: Foot and Ankle Surgery: Official Journal of the European Society of Foot and Ankle Surgeons
https://www.readbyqxmd.com/read/29410724/chronic-skin-fragility-of-aging-current-concepts-in-the-pathogenesis-recognition-and-management-of-dermatoporosis
#3
REVIEW
Joseph M Dyer, Richard A Miller
Thin skin and the appearance of bruises, seemingly unprovoked, are frequent complaints of elderly patients. Chronic cutaneous insufficiencies such as these are termed dermatoporosis. Although it is seldom the primary reason for consultation, dermatoporosis is associated with bleeding and healing complications and presents an opportunity for patient education and prevention. In this review, the authors explore the risk factors, pathogenetic mechanisms, clinical expression, and evidence-based therapies reported for chronic skin fragility due to aging...
January 2018: Journal of Clinical and Aesthetic Dermatology
https://www.readbyqxmd.com/read/29408699/accumulation-of-carboxymethyl-lysine-cml-in-human-cortical-bone
#4
Corinne J Thomas, Timothy P Cleland, Grazyna E Sroga, Deepak Vashishth
Advanced glycation end-products (AGEs) are a category of post translational modification associated with the degradation of the structural properties of multiple different types of tissues. Typically, AGEs are the result of a series of post-translational modification reactions between sugars and proteins through a process known as non-enzymatic glycation (NEG). Increases in the rate of NEG of bone tissue are associated with type 2 diabetes and skeletal fragility. Current methods of assessing NEG and its impact on bone fracture risk involve measurement of pentosidine or total fluorescent AGEs (fAGEs)...
February 1, 2018: Bone
https://www.readbyqxmd.com/read/29391280/injury-and-inflammation-driven-skin-fibrosis-the-paradigm-of-epidermolysis-bullosa
#5
REVIEW
Alexander Nyström, Leena Bruckner-Tuderman
Genetic or acquired destabilization of the dermal extracellular matrix evokes injury- and inflammation-driven progressive soft tissue fibrosis. Dystrophic epidermolysis bullosa (DEB), a heritable human skin fragility disorder, is a paradigmatic disease to investigate these processes. Studies of DEB have generated abundant new information on cellular and molecular mechanisms at play in skin fibrosis which are not only limited to intractable diseases, but also applicable to some of the most common acquired conditions...
January 29, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29346445/transcriptome-analysis-of-skin-fibroblasts-with-dominant-negative-col3a1-mutations-provides-molecular-insights-into-the-etiopathology-of-vascular-ehlers-danlos-syndrome
#6
Nicola Chiarelli, Giulia Carini, Nicoletta Zoppi, Marco Ritelli, Marina Colombi
Vascular Ehlers-Danlos syndrome (vEDS) is a dominantly inherited connective tissue disorder caused by mutations in the COL3A1 gene that encodes type III collagen (COLLIII), which is the major expressed collagen in blood vessels and hollow organs. The majority of disease-causing variants in COL3A1 are glycine substitutions and in-frame splice mutations in the triple helix domain that through a dominant negative effect are associated with the severe clinical spectrum potentially lethal of vEDS, characterized by fragility of soft connective tissues with arterial and organ ruptures...
2018: PloS One
https://www.readbyqxmd.com/read/29339502/protein-aggregation-of-the-p63-transcription-factor-underlies-severe-skin-fragility-in-aec-syndrome
#7
Claudia Russo, Christian Osterburg, Anna Sirico, Dario Antonini, Raffaele Ambrosio, Julia Maren Würz, Jörg Rinnenthal, Marco Ferniani, Sebastian Kehrloesser, Birgit Schäfer, Peter Güntert, Satrajit Sinha, Volker Dötsch, Caterina Missero
The p63 gene encodes a master regulator of epidermal commitment, development, and differentiation. Heterozygous mutations in the C-terminal domain of the p63 gene can cause ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome, a life-threatening disorder characterized by skin fragility and severe, long-lasting skin erosions. Despite deep knowledge of p63 functions, little is known about mechanisms underlying disease pathology and possible treatments. Here, we show that multiple AEC-associated p63 mutations, but not those causative of other diseases, lead to thermodynamic protein destabilization, misfolding, and aggregation, similar to the known p53 gain-of-function mutants found in cancer...
January 16, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29328753/a-critical-analysis-of-the-perioperative-management-of-patients-with-ehlers-danlos-type-iv-vascular-syndrome
#8
Justyna Martin
This article discusses the perioperative care needs of patients with Ehlers Danlos type IV (vascular) syndrome. Ehlers Danlos syndrome (EDS) is a heritable group of connective tissue disorders characterised by varying degrees of tissue, blood vessel and internal organ fragility as well as skin and joint hypermobility (De Paepe and Malfait 2012).
September 2017: Journal of Perioperative Practice
https://www.readbyqxmd.com/read/29326825/staphylococcal-scalded-skin-syndrome-a-pediatric-dermatology-case-report
#9
Girish Gulab Meshram, Neeraj Kaur, Kanwaljeet Singh Hura
Staphylococcal scalded skin syndrome is a condition which predominantly affects children and causes a spectrum of skin lesions. We present a case of a 2-month-old infant with complaints of fever and fragile blisters over the body. The mucosal areas were spared. The diagnosis of staphylococcal scalded skin syndrome was reached on clinical grounds and culture report. The patient responded well to the treatment, which included an antibiotic (cloxacillin), an analgesic (paracetamol), and hydration with intravenous fluids...
2018: SAGE open medical case reports
https://www.readbyqxmd.com/read/29326176/combinatorial-omics-analysis-reveals-perturbed-lysosomal-homeostasis-in-collagen-vii-deficient-keratinocytes
#10
Kerstin Thriene, Björn Andreas Grüning, Olivier Bornert, Anika Erxleben, Juna Leppert, Ioannis Athanasiou, Ekkehard Weber, Dimitra Kiritsi, Alexander Nyström, Thomas Reinheckel, Rolf Backofen, Cristina Has, Leena Bruckner-Tuderman, Joern Dengjel
The extracellular matrix protein collagen VII is part of the microenvironment of stratified epithelia and critical in organismal homeostasis. Mutations in the encoding gene COL7A1 lead to the skin disorder dystrophic epidermolysis bullosa (DEB), are linked to skin fragility and progressive inflammation-driven fibrosis that facilitates aggressive skin cancer. So far, these changes have been linked to mesenchymal alterations, the epithelial consequences of collagen VII loss remaining under-addressed. As epithelial dysfunction is a principal initiator of fibrosis, we performed a comprehensive transcriptome and proteome profiling of primary human keratinocytes to generate global and detailed images of dysregulated epidermal molecular pathways linked to loss of collagen VII...
January 11, 2018: Molecular & Cellular Proteomics: MCP
https://www.readbyqxmd.com/read/29319449/management-and-closure-of-multiple-large-cutaneous-lesions-in-a-juvenile-cat-with-severe-acquired-skin-fragility-syndrome-secondary-to-iatrogenic-hyperadrenocorticism
#11
C Noël McKnight, Linda J Lew, David A Gamble
CASE DESCRIPTION A 13-month-old castrated male cat was evaluated for a large, spontaneously developed cutaneous laceration over the left scapular region. The cat had a history of severe gingivostomatitis, conjunctivitis, giardiasis, and feline herpesvirus infection and had received systemic glucocorticoid treatment for 7 weeks prior to evaluation. CLINICAL FINDINGS Physical examination revealed a 10 × 7-cm full-thickness cutaneous laceration over the left scapular region, extremely thin skin, crusts over the dorsal aspect of the neck and base of the skull, medially curling pinnae, and moderate gingivostomatitis...
January 15, 2018: Journal of the American Veterinary Medical Association
https://www.readbyqxmd.com/read/29305555/impaired-lymphoid-extracellular-matrix-impedes-antibacterial-immunity-in-epidermolysis-bullosa
#12
Alexander Nyström, Olivier Bornert, Tobias Kühl, Christine Gretzmeier, Kerstin Thriene, Jörn Dengjel, Andrea Pfister-Wartha, Dimitra Kiritsi, Leena Bruckner-Tuderman
Genetic loss of collagen VII causes recessive dystrophic epidermolysis bullosa (RDEB), a skin fragility disorder that, unexpectedly, manifests also with elevated colonization of commensal bacteria and frequent wound infections. Here, we describe an unprecedented systemic function of collagen VII as a member of a unique innate immune-supporting multiprotein complex in spleen and lymph nodes. In this complex, collagen VII specifically binds and sequesters the innate immune activator cochlin in the lumen of lymphoid conduits...
January 5, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29247552/ehlers-danlos-syndrome-in-a-young-woman-with-anorexia-nervosa-and-complex-somatic-symptoms
#13
Michelle Lee, Mattias Strand
The Ehler-Danlos syndromes (EDS) are a group of clinically heterogeneous connective tissue disorders characterized by joint hypermobility, hyperextensibility of the skin, and a general connective tissue fragility that can induce symptoms from multiple organ systems. We present a case of comorbid anorexia nervosa and EDS in a 23-year old woman with a multitude of somatic symptoms that were initially attributed to the eating disorder but that were likely caused by the underlying EDS. Various EDS symptoms, such as gastrointestinal complaints, smell and taste abnormalities, and altered somatosensory awareness may resemble or mask an underlying eating disorder, and vice versa...
December 16, 2017: International Journal of Eating Disorders
https://www.readbyqxmd.com/read/29243033/incontinence-associated-dermatitis-pathogenesis-contributing-factors-prevention-and-management-options
#14
Hilde Beele, Steven Smet, Nele Van Damme, Dimitri Beeckman
Incontinence-associated dermatitis (IAD) is a common type of irritant contact dermatitis, seen in patients with urinary or faecal incontinence. Mechanical factors such as traumata and friction may aggravate the lesions. The fragile skin in elderly patients is more prone to developing IAD. The clinical picture of IAD consists of persistent redness on the one hand and skin loss on the other. Both categories may be associated with clinical signs of major colonisation or infection. It is important to distinguish IAD from pressure ulcers and other dermatoses in the genital region...
December 14, 2017: Drugs & Aging
https://www.readbyqxmd.com/read/29210715/blistering-and-skin-fragility-due-to-imatinib-therapy-loss-of-laminin-and-collagen-iv-as-a-possible-cause-of-cutaneous-basement-membrane-instability
#15
Sebastian Mühl, Jan Ehrchen, Dieter Metze
Imatinib mesylate (Glivec; Novartis AG, Basel, Switzerland) is a tyrosine kinase inhibitor which is used in the treatment of oncologic diseases like chronic myeloid leukemia and gastrointestinal stroma tumor (GIST). Among cutaneous side effects, bullous reactions are rare. The authors describe the case of a 66-year-old woman developing blistering and skin fragility on her hands, foot, lower legs, and back after intake of imatinib for treatment of GIST. Biopsy showed vacuolar alteration at the dermoepidermal junction (DEJ) associated with a few lymphocytes and a subepidermal blister...
November 22, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29206923/vascular-abnormalities-in-the-placenta-of-chst14-fetuses-implications-in-the-pathophysiology-of-perinatal-lethality-of-the-murine-model-and-vascular-lesions-in-human-chst14-d4st1-deficiency
#16
Takahiro Yoshizawa, Shuji Mizumoto, Yuki Takahashi, Shin Shimada, Kazuyuki Sugahara, Jun Nakayama, Shin'ichi Takeda, Yoshihiro Nomura, Yuko Nitahara-Kasahara, Takashi Okada, Kiyoshi Matsumoto, Shuhei Yamada, Tomoki Kosho
Collagen is one of the most important components of the extracellular matrix that is involved in the strength of tissues, cell adhesion, and cell proliferation. Mutations in several collagen and post-translational modification enzyme genes cause Ehlers-Danlos syndrome (EDS) characterized by joint and skin hyperextensibility as well as fragility of various organs. Carbohydrate sulfotransferase 14/dermatan 4-O-sulfotransferase-1 (CHST14/D4ST1) is a critical enzyme for biosynthesis of dermatan sulfate, a side chain of various proteoglycans including biglycan that regulates collagen fibrils through their interaction...
December 1, 2017: Glycobiology
https://www.readbyqxmd.com/read/29203033/recessive-dystrophic-epidermolysis-bullosa-and-pregnancy
#17
F Boria, R Maseda, M Martín-Cameán, M De la Calle, R de Lucas
Dystrophic epidermolysis bullosa is a rare inherited disease caused by mutations in the COL7A1 gene. Its recessive variant (recessive dystrophic epidermolysis bullosa) is characterized by the absence or considerably reduced expression of type VII collagen, which leads to marked fragility of the skin and mucous membranes and subsequent blister formation, whether spontaneously or following minimal injury. There have been very few reports of this disease in pregnant women. We present 2 cases of pregnant women with recessive dystrophic epidermolysis bullosa managed in our High-Risk Pregnancy Unit at Hospital Universitario La Paz, Madrid, Spain...
December 1, 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/29197867/topical-coconut-oil-in-very-preterm-infants-an-open-label-randomised-controlled-trial
#18
Tobias Strunk, Sameer Pupala, Julie Hibbert, Dorota Doherty, Sanjay Patole
BACKGROUND: The immature fragile skin of preterm infants represents an inadequate protective barrier. The emollient and anti-infective properties of coconut oil make it a potentially beneficial topical agent for this population. OBJECTIVES: Our aim was to evaluate feasibility, safety, and the effects of topical coconut oil on skin condition in very preterm infants. METHODS: An open-label randomised controlled trial in preterm infants <30 weeks' gestation was conducted...
2018: Neonatology
https://www.readbyqxmd.com/read/29192600/magazines-in-waiting-areas-of-hospital-a-forgotten-microbial-reservoir
#19
Mathias Adé, Sandrine Burger, Anaelle Cuntzmann, Julien Exinger, Olivier Meunier
The hospital environment is a potential source of microbial contamination. Thus, the magazines in hospital's waiting rooms are handled by patients and visitors whose health and hygiene conditions can vary widely. In this context, we had measured the microbial load on the surface of magazines. Fifteen magazines from 5 waiting rooms of hospital are sampled by agar prints at the areas taken in hand. The agar plates are incubated at 30̊C for 72h. The colonies are counted and identified by MALDI-TOF mass spectrometry (Vitek®-MS)...
December 1, 2017: Annales de Biologie Clinique
https://www.readbyqxmd.com/read/29180129/genetic-diagnosis-of-epidermolysis-bullosa-recommendations-from-an-expert-spanish-research-group
#20
C Sánchez-Jimeno, M J Escámez, C Ayuso, M J Trujillo-Tiebas, M Del Río
Epidermolysis bullosa (EB) is a rare genetic disease that causes mucocutaneous fragility. It comprises a clinically and genetically heterogeneous group of disorder characterized by spontaneous or contact/friction-induced blistering. EB is classified into 4 types-simplex, junctional, dystrophic, and Kindler syndrome-and 30 subtypes. The disease is caused by defects in proteins implicated in dermal-epidermal adhesion. At least 19 genes have been characterized and more than 1000 mutations identified, thus rendering diagnosis complex...
November 24, 2017: Actas Dermo-sifiliográficas
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