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Inflammatory Linear Verrucous Epidermal Nevus (Ilven)

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Neurocutaneous Disorders Phakomatoses and Hamartoneoplastic Syndromes

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Abstract

The term ILVEN (inflammatory linear verrucous epidermal nevus) describes a distinct variety of keratinocytic epidermal nevus which presents as linear, pruritic, erythematous, and hyperkeratotic papules that often coalesce into plaques, with a raised scaly surface, occurring unilaterally in narrow linear patterns following the lines of Blaschko (Lee and Rogers 2001, Rogers 2006, Sugarman 2004, Vujevich and Mancini 2004). The nevus can be present at birth, but most frequently arises in infancy (Lee and Rogers 2001). It appears clinically as inflammatory (Rogers 2006) but histologically demonstrates ortho-hyperkeratosis alternating with parakeratotic hyperkeratosis. Affected patients are generally otherwise normal but there have been multiple reports of ILVEN associated with ipsilateral skeletal abnormalities (often representing incorrectly diagnosed cases of CHILD syndrome) (Lee and Rogers 2001, Rogers 2006) and isolated case reports in association with systemic and/or nervous system malformations and neurological dysfunction.

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Author information

Authors and Affiliations

  1. Institute of Neurological Science, National Research Council, Catania and Department of Paediatrics, University of Catania, Catania, Italy

    Martino Ruggieri

  2. Department of Dermatology and Community and Family Medicine, University of California, San Francisco, USA

    Jeffrey L. Sugarman

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  1. Martino Ruggieri
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  2. Jeffrey L. Sugarman
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Editor information

Editors and Affiliations

  1. Institute of Neurological Science, National Research Council, Catania, Italy

    Martino Ruggieri

  2. University Hospital La Paz, Madrid, Spain

    Ignacio Pascual-Castroviejo

  3. Institute of Neurosurgery, Catholic University of the Sacred Heart, Rome, Italy

    Concezio Di Rocco

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Ruggieri, M., Sugarman, J.L. (2008). Inflammatory Linear Verrucous Epidermal Nevus (Ilven). In: Ruggieri, M., Pascual-Castroviejo, I., Di Rocco, C. (eds) Neurocutaneous Disorders Phakomatoses and Hamartoneoplastic Syndromes. Springer, Vienna. https://doi.org/10.1007/978-3-211-69500-5_34

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  • DOI: https://doi.org/10.1007/978-3-211-69500-5_34

  • Publisher Name: Springer, Vienna

  • Print ISBN: 978-3-211-21396-4

  • Online ISBN: 978-3-211-69500-5

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