Plectin in Skin Fragility Disorders

Plectin is a multi-faceted, 500 kDa-large protein, which due to its expression in different isoforms and distinct organs acts diversely as a cytoskeletal crosslinker and signaling scaffold. It functions as a mediator of keratinocyte mechanical stability in the skin, primarily through linking intermediate filaments to hemidesmosomes. Skin fragility may occur through the presence of mutations in the gene encoding for plectin, PLEC, or through the presence of autoantibodies against the molecule. Below, we review the cutaneous manifestations of plectinopathies as well as their systemic involvement in specific disease subtypes. We summarize the known roles of plectin in keratinocytes and fibroblasts and provide an outlook on future perspectives for plectin-associated skin disorders.

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Atypical and Rare Forms of Cutaneous Lupus Erythematosus: The Importance of the Diagnosis for the Best Management of Patients

Dermatology ◽

10.1159/000515766 ◽

2021 ◽

pp. 1-10

Author(s):

Astrid Herzum ◽

Giulia Gasparini ◽

Emanuele Cozzani ◽

Martina Burlando ◽

Aurora Parodi

Keyword(s):

General Practitioner ◽

Autoimmune Disease ◽

Lupus Erythematosus ◽

Cutaneous Lupus Erythematosus ◽

Cutaneous Manifestations ◽

Skin Manifestation ◽

Systemic Involvement ◽

Best Management ◽

Wide Range ◽

Cutaneous Lupus

Lupus erythematosus (LE) is an autoimmune disease with a wide range of clinical and cutaneous manifestations. Along with the well-known typical cutaneous manifestations of LE, some cutaneous manifestations are rarer, but still characteristic, enabling the dermatologist and the general practitioner who know them to suspect cutaneous LE (CLE) and investigate a possible underlying systemic involvement. Indeed, not infrequently a skin manifestation is the first presentation of systemic LE (SLE), and >75% of SLE patients show signs of skin disease during the course of the illness. Especially, SLE involvement occurs in cases of acute CLE, while it is uncommon in subacute CLE and rare in chronic CLE. This review aims to concentrate especially on atypical cutaneous manifestations of LE to enable the clinician to diagnose even the rarest forms of CLE.

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Recent advanced applications of ion-gel in ionic-gated transistor

npj Flexible Electronics ◽

10.1038/s41528-021-00110-2 ◽

2021 ◽

Vol 5 (1) ◽

Author(s):

Depeng Wang ◽

Shufang Zhao ◽

Ruiyang Yin ◽

Linlin Li ◽

Zheng Lou ◽

...

Keyword(s):

Recent Progress ◽

Mechanical Stability ◽

Dielectric Material ◽

Charge Carriers ◽

Future Perspectives ◽

Organic Transistor ◽

Ion Gel ◽

Physical And Chemical ◽

Large Capacitance ◽

Advanced Applications

AbstractDiversified regulation of electrons have received much attention to realize a multi-functional transistor, and it is crucial to have a considerable control over the charge carriers in transistors. Ionic gel, as the dielectric material in transistors, facilitates a large capacitance, and high induced-carrier concentrations. This review presents the recent progress in ionic-gated transistors (IGTs) that have good mechanical stability as well as high physical and chemical stability. We first briefly introduce the various applications of IGTs in sensors, neuromorphic transistors, organic transistor circuits, and health detection. Finally, the future perspectives of IGTs are discussed and some possible solutions to the challenges are also proposed.

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Skin Manifestations Associated with COVID-19: Current Knowledge and Future Perspectives

Dermatology ◽

10.1159/000512932 ◽

2020 ◽

pp. 1-12 ◽

Cited By ~ 1

Author(s):

Giovanni Genovese ◽

Chiara Moltrasio ◽

Emilio Berti ◽

Angelo Valerio Marzano

Keyword(s):

Severe Acute Respiratory Syndrome ◽

Clinical Features ◽

Current Knowledge ◽

Future Perspectives ◽

Cutaneous Manifestations ◽

Skin Manifestations ◽

Global Pandemic ◽

Morbilliform Rash ◽

First Time ◽

Polymorphic Nature

Background: Coronavirus disease-19 (COVID-19) is an ongoing global pandemic caused by the “severe acute respiratory syndrome coronavirus 2” (SARS-CoV-2), which was isolated for the first time in Wuhan (China) in December 2019. Common symptoms include fever, cough, fatigue, dyspnea and hypogeusia/hyposmia. Among extrapulmonary signs associated with COVID-19, dermatological manifestations have been increasingly reported in the last few months. Summary: The polymorphic nature of COVID-19-associated cutaneous manifestations led our group to propose a classification, which distinguishes the following six main clinical patterns: (i) urticarial rash, (ii) confluent erythematous/maculopapular/morbilliform rash, (iii) papulovesicular exanthem, (iv) chilblain-like acral pattern, (v) livedo reticularis/racemosa-like pattern, (vi) purpuric “vasculitic” pattern. This review summarizes the current knowledge on COVID-19-associated cutaneous manifestations, focusing on clinical features and therapeutic management of each category and attempting to give an overview of the hypothesized pathophysiological mechanisms of these conditions.

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Barrier-Restoring Therapies in Atopic Dermatitis: Current Approaches and Future Perspectives

Dermatology Research and Practice ◽

10.1155/2012/923134 ◽

2012 ◽

Vol 2012 ◽

pp. 1-6 ◽

Cited By ~ 19

Author(s):

Y. Valdman-Grinshpoun ◽

D. Ben-Amitai ◽

A. Zvulunov

Keyword(s):

Atopic Dermatitis ◽

Inflammatory Disease ◽

Clinical Studies ◽

Skin Barrier ◽

Future Perspectives ◽

Barrier Dysfunction ◽

Gene Encoding ◽

Promising Strategy ◽

Topical Treatments ◽

Barrier Repair

Atopic dermatitis is a multifactorial, chronic relapsing, inflammatory disease, characterized by xerosis, eczematous lesions, and pruritus. The latter usually leads to an “itch-scratch” cycle that may compromise the epidermal barrier. Skin barrier abnormalities in atopic dermatitis may result from mutations in the gene encoding for filaggrin, which plays an important role in the formation of cornified cytosol. Barrier abnormalities render the skin more permeable to irritants, allergens, and microorganisms. Treatment of atopic dermatitis must be directed to control the itching, suppress the inflammation, and restore the skin barrier. Emollients, both creams and ointments, improve the barrier function of stratum corneum by providing it with water and lipids. Studies on atopic dermatitis and barrier repair treatment show that adequate lipid replacement therapy reduces the inflammation and restores epidermal function. Efforts directed to develop immunomodulators that interfere with cytokine-induced skin barrier dysfunction, provide a promising strategy for treatment of atopic dermatitis. Moreover, an impressive proliferation of more than 80 clinical studies focusing on topical treatments in atopic dermatitis led to growing expectations for better therapies.

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Dermatological Manifestations of COVID-19 in Children

Journal of Skin and Stem Cell ◽

10.5812/jssc.106890 ◽

2020 ◽

Vol 7 (2) ◽

Author(s):

Vivek Athwani ◽

Sunil Gothwal

Keyword(s):

Toxic Shock Syndrome ◽

Gastrointestinal Symptoms ◽

Skin Lesions ◽

Skin Involvement ◽

Toxic Shock ◽

Cutaneous Manifestations ◽

Systemic Involvement ◽

Atypical Kawasaki Disease ◽

Coronavirus Infection ◽

Inflammatory Syndrome

: Coronavirus infection 2019 (COVID-19) primarily has a respiratory system and multi-systemic involvement. Respiratory and gastrointestinal symptoms are predominantly seen in children. In adults, few COVID-19 cases are reported with cutaneous manifestations. Although children are less severely affected by COVID-19, there is increasing evidence for skin involvement, which is in the form of chilblain (e.g., lesions, vesicular, and maculopapular) and erythema multiforme (e.g., rash). Also, few COVID-19 cases are presented with a clinical picture of atypical Kawasaki disease and toxic shock syndrome, later defined as pediatric multisystem inflammatory syndrome (PMIS). The present study aims to summarize various skin lesions with COVID-19.

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Psoriasis in HIV infection: an update

International Journal of STD & AIDS ◽

10.1177/0956462419827673 ◽

2019 ◽

Vol 30 (6) ◽

pp. 596-604

Author(s):

Miguel Alpalhão ◽

J. Borges-Costa ◽

Paulo Filipe

Keyword(s):

Drug Therapy ◽

Hiv Infection ◽

Clinical Features ◽

Clinical Presentation ◽

Treatment Options ◽

Future Perspectives ◽

Biological Drugs ◽

Cutaneous Manifestations ◽

Immune Mediated ◽

Novel Drugs

Psoriasis is a prevalent systemic immune-mediated disease with cutaneous manifestations. In HIV-infected patients, psoriasis may have a higher incidence, present atypical and more exuberant clinical features, and is frequently recalcitrant to treatment. Despite this aggravated severity, treatment options for psoriasis in HIV-infected individuals remain limited due to the risk of fatal immunosuppression associated with both classical immunosuppressants and new biological drugs. Notwithstanding, drug therapy in psoriasis has been undergoing major advances for the last few years, with novel drugs approved, which could significantly add to the management of HIV-infected patients. It is therefore our aim to present a review of the available literature to highlight the updated evidence on psoriasis in HIV-infected individuals, particularly in regards to its epidemiology, proposed pathophysiology, clinical presentation, currently available therapeutic options, and future perspectives.

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Skin manifestations in spondyloarthritis

Therapeutic Advances in Musculoskeletal Disease ◽

10.1177/1759720x20975915 ◽

2020 ◽

Vol 12 ◽

pp. 1759720X2097591

Author(s):

Katharina Meier ◽

Alexandra Schloegl ◽

Denis Poddubnyy ◽

Kamran Ghoreschi

Keyword(s):

Skin Diseases ◽

Joint Involvement ◽

Skin Disorders ◽

Psychosocial Burden ◽

Cutaneous Manifestations ◽

Skin Manifestations ◽

Plaque Type ◽

Interdisciplinary Field ◽

Clinical Presentations ◽

Physical Impact

Spondyloarthritides (SpA) like psoriatic arthritis, axial spondyloarthritis/ankylosing spondylitis, reactive arthritis and inflammatory bowel disease (IBD)-associated SpA can present with characteristic skin manifestations. These SpA-associated skin disorders may precede joint involvement, reflect a loss of efficacy of a current systemic treatment or can even be treatment associated. Cutaneous manifestations in SpA not only add additional morbidity with physical impact but also impose a psychosocial burden on affected patients. Psoriasis (PsO) – the main skin disease in SpA – has a variety of clinical presentations, including plaque-type PsO, inverse PsO, guttate PsO, erythrodermic PsO, nail PsO and pustular types. SpA associated with IBD presents with neutrophilic and granulomatous skin disorders, including pyoderma gangrenosum, hidradenitis suppurativa and cutaneous Crohn’s disease. Reactive arthritides has a favourable prognosis and may feature keratoderma blenorrhagicum or balanitis circinatum as typical skin manifestations. Immunologically, SpA-associated skin diseases share interleukin (IL)-17 and IL-23 dysregulation but show distinctive genetic and immunological profiles. Therefore, they vary in their treatment responses to targeted therapies with biologicals or small molecules. In this review, we highlight the clinical presentation of skin manifestations in SpA and discuss therapeutic approaches in this interdisciplinary field.

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A crystallization technique for obtaining large protein crystals with increased mechanical stability using agarose gel combined with a stirring technique

Journal of Crystal Growth ◽

10.1016/j.jcrysgro.2015.11.008 ◽

2016 ◽

Vol 452 ◽

pp. 172-178 ◽

Cited By ~ 5

Author(s):

Mihoko Maruyama ◽

Yuki Hayashi ◽

Hiroshi Y. Yoshikawa ◽

Shino Okada ◽

Haruhiko Koizumi ◽

...

Keyword(s):

Mechanical Stability ◽

Protein Crystals ◽

Agarose Gel ◽

Large Protein

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A Familial Form of Epidermolysis Bullosa Simplex Associated with a Pathogenic Variant in KRT5

Genes ◽

10.3390/genes12101503 ◽

2021 ◽

Vol 12 (10) ◽

pp. 1503

Author(s):

Francesco Paduano ◽

Emma Colao ◽

Teresa Grillone ◽

Marco Flavio Michele Vismara ◽

Rosario Amato ◽

...

Keyword(s):

Epidermolysis Bullosa ◽

Mechanical Stability ◽

Pathogenic Variant ◽

Mechanical Trauma ◽

Epidermolysis Bullosa Simplex ◽

Pathogenic Variants ◽

Family Pedigree ◽

Familial Form ◽

The Family ◽

Skin Fragility

Epidermolysis bullosa simplex is a disease that belongs to a group of genodermatoses characterised by the formation of superficial bullous lesions caused by minor mechanical trauma to the skin. The skin fragility observed in the EBS is mainly caused by pathogenic variants in the KRT5 and KRT14 genes that compromise the mechanical stability of epithelial cells. By performing DNA sequencing in a female patient with EBS, we found the pathogenic variant c.967G>A (p.Val323Met) in the KRT5 gene. This variant co-segregated with EBS in the family pedigree and was transmitted in an autosomal dominant inheritance manner. This is the first report showing a familial form of EBS due to this pathogenic variant.

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PATTERN OF CUTANEOUS MANIFESTATIONS OF DIABETES MELLITUS

10.36106/paripex/6900256 ◽

2020 ◽

pp. 1-3

Author(s):

Satpal Yadav ◽

Aman Goyal ◽

Poonam Verma

Keyword(s):

Diabetes Mellitus ◽

Acanthosis Nigricans ◽

Cutaneous Manifestation ◽

Skin Disorders ◽

Cutaneous Manifestations ◽

Cutaneous Infections ◽

Endocrine Disorder ◽

Study Results ◽

Common Skin ◽

The Common

BACKGROUND: Diabetes is most common endocrine disorder with increasing prevalence worldwide. Every organs system is affected by it albeit at different pace. This study was carried out with the aim of studying cutaneous manifestation of diabetes mellitus. METHODS- 200 cases of diabetes mellitus with cutaneous manifestations attending out patient department of dermatology were included in the study. RESULTS - The common skin disorders were cutaneous infections (53.5%), acanthosis nigricans (10.5%), generalized pruritus (10.5%),skintags (9.5%). CONCLUSION - most common dermatological manifestations were cutaneous infections.

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