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Hereditary Disorders of the Dermis

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Therapy in Pediatric Dermatology

Abstract

Various proteins compose the connective tissue that is found in normal skin and account for its strength and elasticity. Abnormalities in the structure or function of any of these essential dermal proteins can lead to a myriad of highly variable cutaneous disorders, and because some of these same proteins that are important in the skin also play a critical role in the connective tissue of blood vessels and other organs, these entities range in severity, from entirely benign to those that have significant morbidity or are even lethal. Although occasionally acquired, the majority of the disorders of the dermis are inherited, and the recent discovery of many of the causative mutations has led to improved classification schemes and a clearer understanding of their pathogenesis. Medical therapies for these hereditary disorders of the dermis are limited, and in many cases, optimal medical management of these conditions consists instead of providing an accurate diagnosis, implementation of preventative strategies to avoid future complications, and a multidisciplinary approach to address the cutaneous and extra cutaneous manifestations.

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Author information

Authors and Affiliations

  1. Dermatology and Pediatric & Adolescent Medicine, Mayo Clinic, Rochester, MN, USA

    Megha M. Tollefson

  2. Department of Pediatrics, The Ohio State University, Columbus, OH, USA

    Katya L. Harfmann

  3. Dermatology and Pediatrics, University of Mississippi Medical Center, Jackson, MS, USA

    Stephanie K. Jacks

  4. Division and Section of Dermatology, Department of Pediatrics, Nationwide Children’s Hospital, The Ohio State University, Columbus, OH, USA

    Patricia M. Witman

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  1. Megha M. Tollefson
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  2. Katya L. Harfmann
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  3. Stephanie K. Jacks
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  4. Patricia M. Witman
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Correspondence to Patricia M. Witman .

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Editors and Affiliations

  1. Stanford University School of Medicine, Stanford, California, USA

    Joyce M.C. Teng

  2. Stanford University School of Medicine, Stanford, California, USA

    Ann L. Marqueling

  3. Pediatric Dermatology, Joe DiMaggio Children’s Hospital, Hollywood, Florida, USA

    Latanya T. Benjamin

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Tollefson, M.M., Harfmann, K.L., Jacks, S.K., Witman, P.M. (2017). Hereditary Disorders of the Dermis. In: Teng, J., Marqueling, A., Benjamin, L. (eds) Therapy in Pediatric Dermatology. Springer, Cham. https://doi.org/10.1007/978-3-319-43630-2_6

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