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Epidermolytic Ichthyosis

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Clinical Cases in Neonatal and Infant Dermatology

Part of the book series: Clinical Cases in Dermatology ((CLIDADE))

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Abstract

Ichthyosis is a group of genodermatosis characterized by desquamation disorders in the form of hyperkeratosis and generalized scale. Epidermolytic ichthyosis (EI) is an autosomal dominant hereditary disorder caused by mutations in keratin 1 or 10 genes. Clinical manifestations of EI are bullae, erythroderma, scales with accentuated pattern in the body folds area, and palmoplantar hyperkeratosis. Histopathological examination of EI showed hyperkeratosis, parakeratosis, vacuolization of keratinocytes, increased keratohyalin, and intraepidermal bullae. Clinical manifestation of EI could resemble many skin disorders, however with careful history taking and histopathological examination could establish the diagnosis.

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Author information

Authors and Affiliations

  1. Department of Dermatology and Venereology, Pediatric Dermatology Division, Faculty of Medicine, Dr. Hasan Sadikin Hospital, Universitas Padjadjaran, Bandung, Indonesia

    Reiva Farah Dwiyana, Reyshiani Johan, Raden Mohamad Rendy Ariezal Effendi, Srie Prihianti Gondokaryono & Inne Arline Diana

  2. Indonesian Pediatric Dermatology Study Group, Indonesian Society of Dermatology and Venereology, Jakarta, Indonesia

    Reiva Farah Dwiyana, Reyshiani Johan, Raden Mohamad Rendy Ariezal Effendi, Srie Prihianti Gondokaryono & Inne Arline Diana

Authors
  1. Reiva Farah Dwiyana
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  2. Reyshiani Johan
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  3. Raden Mohamad Rendy Ariezal Effendi
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  4. Srie Prihianti Gondokaryono
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  5. Inne Arline Diana
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Correspondence to Reiva Farah Dwiyana .

Editor information

Editors and Affiliations

  1. Dermatology, University of Rome “G. Marconi”, Rome, Italy

    Fabio Arcangeli

  2. Dermatology, University of Rome “G. Marconi”, Rome, Italy

    Torello M. Lotti

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Dwiyana, R.F., Johan, R., Effendi, R.M.R.A., Gondokaryono, S.P., Diana, I.A. (2022). Epidermolytic Ichthyosis. In: Arcangeli, F., Lotti, T.M. (eds) Clinical Cases in Neonatal and Infant Dermatology. Clinical Cases in Dermatology. Springer, Cham. https://doi.org/10.1007/978-3-030-91523-0_23

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  • DOI: https://doi.org/10.1007/978-3-030-91523-0_23

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-91522-3

  • Online ISBN: 978-3-030-91523-0

  • eBook Packages: MedicineMedicine (R0)

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