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  1. Article ; Online: Entzündliche Dermatosen im Kindes- und Jugendalter : Diagnostische und therapeutische Besonderheiten anhand der atopischen Dermatitis und der Psoriasis.

    Süßmuth, Kira / Magnolo, Nina / Oji, Vinzenz / Koll, Phillipp Otto / Striegel, Anne / Tantcheva-Poór, Iliana

    Dermatologie (Heidelberg, Germany)

    2024  Volume 75, Issue 4, Page(s) 325–339

    Abstract: Atopic dermatitis (AD) and psoriasis belong to the most common inflammatory dermatoses that we treat in everyday clinical practice. AD manifests in more than 70% of cases before the age of 5 years. Approximately one-third of psoriasis patients report on ... ...

    Title translation Inflammatory dermatoses in children and adolescents : Diagnosis and treatment of atopic dermatitis and psoriasis.
    Abstract Atopic dermatitis (AD) and psoriasis belong to the most common inflammatory dermatoses that we treat in everyday clinical practice. AD manifests in more than 70% of cases before the age of 5 years. Approximately one-third of psoriasis patients report on onset of disease in the first two decades of life. Here, we are going to review both disorders in the light of pediatric dermatology. We are going to discuss selected subtypes and present clues for further examination with respect to the differential diagnoses and comorbidities. The article provides insight into current therapeutic developments that are relevant for the treatment of children and adolescents.
    MeSH term(s) Adolescent ; Child ; Child, Preschool ; Humans ; Dermatitis, Atopic/diagnosis ; Diagnosis, Differential ; Psoriasis/diagnosis
    Language German
    Publishing date 2024-02-14
    Publishing country Germany
    Document type English Abstract ; Journal Article ; Review
    ISSN 2731-7013
    ISSN (online) 2731-7013
    DOI 10.1007/s00105-023-05257-9
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: Häufige Ichthyosen im Kindesalter. Ichthyosis vulgaris von X-chromosomal rezessiver Ichthyose unterscheiden

    Oji, Vinzenz

    Hautnah : Dermatologie : Dermatologie aus der Praxis

    2017  Volume 33, Issue 5, Page(s) 40

    Language German
    Document type Article
    ZDB-ID 1058402-x
    ISSN 0938-0221 ; 0930-7109
    Database Current Contents Medicine

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    In stock of ZB MED Cologne/Königswinter

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  3. Article ; Online: [No title information]

    Süßmuth, Kira / Traupe, Heiko / Metze, Dieter / Oji, Vinzenz

    Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG

    2020  Volume 18, Issue 3, Page(s) 225–245

    Title translation Ichthyosen im klinischen Alltag: Umgang mit einer seltenen Erkrankungsgruppe.
    Language German
    Publishing date 2020-03-04
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 2093479-8
    ISSN 1610-0387 ; 1610-0379
    ISSN (online) 1610-0387
    ISSN 1610-0379
    DOI 10.1111/ddg.14049_g
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  4. Article ; Online: The Fate of Epidermal Tight Junctions in the

    Haftek, Marek / Oji, Vinzenz / Feldmeyer, Laurence / Hohl, Daniel / Hadj-Rabia, Smaïl / Abdayem, Rawad

    International journal of molecular sciences

    2022  Volume 23, Issue 13

    Abstract: We evaluated the presence of tight junction (TJ) remnants in ... ...

    Abstract We evaluated the presence of tight junction (TJ) remnants in the
    MeSH term(s) Alopecia ; Cholangitis, Sclerosing ; Claudin-1/deficiency ; Epidermal Cells ; Epidermis/metabolism ; Humans ; Ichthyosis ; Leukocyte Disorders ; Skin Diseases/metabolism ; Tight Junctions/metabolism
    Chemical Substances Claudin-1
    Language English
    Publishing date 2022-07-05
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms23137486
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Erythrokeratodermia Variabilis-like Phenotype in Patients Carrying

    Hotz, Alrun / Fölster-Holst, Regina / Oji, Vinzenz / Bourrat, Emmanuelle / Frank, Jorge / Marrakchi, Slaheddine / Ennouri, Mariem / Wankner, Lotta / Komlosi, Katalin / Alter, Svenja / Fischer, Judith

    Genes

    2024  Volume 15, Issue 3

    Abstract: Erythrokeratodermia variabilis (EKV) is a rare genodermatosis characterized by well-demarcated erythematous patches and hyperkeratotic plaques. EKV is most often transmitted in an autosomal dominant manner. Until recently, only mutations in connexins ... ...

    Abstract Erythrokeratodermia variabilis (EKV) is a rare genodermatosis characterized by well-demarcated erythematous patches and hyperkeratotic plaques. EKV is most often transmitted in an autosomal dominant manner. Until recently, only mutations in connexins such as
    MeSH term(s) Humans ; Erythrokeratodermia Variabilis/genetics ; Mutation ; Phenotype ; Pedigree ; ATP-Binding Cassette Transporters/genetics
    Chemical Substances ABCA12 protein, human ; ATP-Binding Cassette Transporters
    Language English
    Publishing date 2024-02-24
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2527218-4
    ISSN 2073-4425 ; 2073-4425
    ISSN (online) 2073-4425
    ISSN 2073-4425
    DOI 10.3390/genes15030288
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article: Häufige Ichthyosen im Kindesalter. Ichthyosis vulgaris von X-chromosomal rezessiver Ichthyose unterscheiden

    Oji, Vinzenz

    Pädiatrie

    2015  Volume 27, Issue S1 Sonderheft Dermatologie, Page(s) 6

    Language German
    Document type Article
    ZDB-ID 2440229-1
    ISSN 1867-2132 ; 1437-1782
    Database Current Contents Medicine

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 2867: Show issues Location:
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  7. Article ; Online: Ichthyoses in everyday practice: management of a rare group of diseases.

    Süßmuth, Kira / Traupe, Heiko / Metze, Dieter / Oji, Vinzenz

    Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG

    2020  Volume 18, Issue 3, Page(s) 225–243

    Abstract: Ichthyoses comprise a heterogeneous group of hereditary disorders of keratinization characterized by a highly varied clinical picture. A distinction is made between common hereditary ichthyoses (ichthyosis vulgaris and X-linked ichthyosis), which usually ...

    Abstract Ichthyoses comprise a heterogeneous group of hereditary disorders of keratinization characterized by a highly varied clinical picture. A distinction is made between common hereditary ichthyoses (ichthyosis vulgaris and X-linked ichthyosis), which usually manifest themselves in the first year of life, and rare, sometimes severe congenital ichthyoses. Patients with very mild symptoms often do not even realize they have ichthyosis. The diagnosis is usually based on clinical evaluation. Molecular genetic testing as well as histological and electron microscopic studies may aid in confirming the diagnosis. Mapping a family tree is also diagnostically useful. Besides skin manifestations, important aspects of the clinical examination and history include disease onset, presence of a collodion membrane at birth as well as the presence of hair anomalies and extracutaneous signs and symptoms. Rigorous hydration of the skin (several times a day) and balneotherapy are the mainstay of ichthyosis treatment. For patients with severe disease, systemic acitretin treatment should be considered on a case-by-case basis. While ichthyoses are generally limited to the skin, there are syndromic forms that may affect other organs and that require interdisciplinarity cooperation. Although ichthyoses remain incurable, they can be managed well with symptomatic treatment. However, such treatment is frequently time consuming and expensive. In the future, novel therapeutic approaches might include enzyme replacement and gene therapies as well as antiinflammatory drugs.
    MeSH term(s) Diagnosis, Differential ; Female ; Genetic Testing ; Humans ; Ichthyosis/diagnosis ; Male ; Physical Examination
    Language English
    Publishing date 2020-03-02
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 2093479-8
    ISSN 1610-0387 ; 1610-0379
    ISSN (online) 1610-0387
    ISSN 1610-0379
    DOI 10.1111/ddg.14049
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Autosomal dominant lamellar ichthyosis due to a missense mutation in the gene NKPD1.

    Komlosi, Katalin / Glocker, Cristina / Hsu-Rehder, Hao-Hsiang / Alter, Svenja / Kopp, Julia / Hotz, Alrun / Zimmer, Andreas David / Hausser-Siller, Ingrid / Sandhoff, Roger / Oji, Vinzenz / Fischer, Judith

    The Journal of investigative dermatology

    2024  

    Abstract: The identification of monogenic causes for cornification disorders has enhanced our understanding of epidermal differentiation and skin barrier function. Autosomal dominant lamellar ichthyosis (ADLI) is a rare condition, and ASPRV1 was the only gene ... ...

    Abstract The identification of monogenic causes for cornification disorders has enhanced our understanding of epidermal differentiation and skin barrier function. Autosomal dominant lamellar ichthyosis (ADLI) is a rare condition, and ASPRV1 was the only gene linked to ADLI to date. We identified a heterozygous variant (ENST00000686631.1:c.1372G>T, p.(Val458Phe)) in the NKPD1 gene in seven individuals from a four-generation German pedigree with generalized lamellar ichthyosis by whole exome sequencing. Segregation analysis confirmed its presence in affected individuals, resulting in a LOD score of 3.31. NKPD1 encodes the NTPase KAP Family P-Loop Domain-Containing Protein 1, implicated in the plasma membrane, its role in human disease is as yet unknown. Skin histology showed moderate acanthosis and compact orthohyperkeratosis, and the ultrastructure differed clearly from that in ASPRV1-ADLI. While NKPD1 mRNA expression increased during keratinocyte differentiation, stratum corneum ceramides exhibited no significant changes. However, affected individuals showed an elevated ratio of protein-bound ceramides to omega-esterified ceramides. This highlights NKPD1's role in ADLI, impacting ceramide metabolism and skin lipid barrier formation, as demonstrated through functional characterization.
    Language English
    Publishing date 2024-04-18
    Publishing country United States
    Document type Journal Article
    ZDB-ID 80136-7
    ISSN 1523-1747 ; 0022-202X
    ISSN (online) 1523-1747
    ISSN 0022-202X
    DOI 10.1016/j.jid.2024.03.041
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: The Fate of Epidermal Tight Junctions in the stratum corneum

    Marek Haftek / Vinzenz Oji / Laurence Feldmeyer / Daniel Hohl / Smaïl Hadj-Rabia / Rawad Abdayem

    International Journal of Molecular Sciences, Vol 23, Iss 7486, p

    Their Involvement in the Regulation of Desquamation and Phenotypic Expression of Certain Skin Conditions

    2022  Volume 7486

    Abstract: We evaluated the presence of tight junction (TJ) remnants in the stratum corneum (SC) of in vitro reconstructed human epidermis and human skin explants subjected or not to an aggressive topical treatment with beta-lipohydroxy salicylic acid (LSA) for 24 ... ...

    Abstract We evaluated the presence of tight junction (TJ) remnants in the stratum corneum (SC) of in vitro reconstructed human epidermis and human skin explants subjected or not to an aggressive topical treatment with beta-lipohydroxy salicylic acid (LSA) for 24 h. LSA-treated samples showed an increased presence of TJ remnants in the two lowermost layers of the SC, as quantified with standard electron microscopy. The topical aggression-induced overexpression of TJ-like cell–cell envelope fusions may influence SC functions: (1) directly, through an enhanced cohesion, and (2) indirectly, by impeding accessibility of peripheral corneodesmosomes to extracellular hydrolytic enzymes and, thus, slowing down desquamation. Observations of ichthyotic epidermis in peeling skin disease (PSD; corneodesmosin deficiency; two cases) and ichthyosis hypotrichosis sclerosing cholangitis syndrome (IHSC/NISCH; absence of claudin-1; two cases) also demonstrated increased persistence of TJ-like intercellular fusions in pathological SC and contributed to the interpretation of the diseases’ pathological mechanisms.
    Keywords tight junction ; stratum corneum ; desquamation ; peeling skin disease (PSD) ; ichthyosis hypotrichosis sclerosing cholangitis (IHSC/NISCH) syndrome ; ultrastructure ; Biology (General) ; QH301-705.5 ; Chemistry ; QD1-999
    Subject code 571
    Language English
    Publishing date 2022-07-01T00:00:00Z
    Publisher MDPI AG
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  10. Article ; Online: Rezidivierendes akutes hämorrhagisches Ödem des Kindesalters (AHEI) in der Pubertät.

    Drerup, Christian / Hake, Lisanne / Oji, Vinzenz / Sunderkötter, Cord

    Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG

    2018  Volume 16, Issue 12, Page(s) 1496–1498

    Language English
    Publishing date 2018-12-28
    Publishing country Germany
    Document type Letter
    ZDB-ID 2093479-8
    ISSN 1610-0387 ; 1610-0379
    ISSN (online) 1610-0387
    ISSN 1610-0379
    DOI 10.1111/ddg.13699_g
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