Citation, DOI, disclosures and article data
Citation:
Bell D, Ichthyosis. Reference article, Radiopaedia.org (Accessed on 25 Apr 2024) https://doi.org/10.53347/rID-60025
Ichthyosis is a heterogeneous set of dermatologic conditions marked out by their phenotype of diffuse dryness of the skin. Ichthyosis is usually inherited, but rarely maybe acquired.
Inherited ichthyosis (II) accounts for the majority of cases. Ichthyosis vulgaris, the commonest form has an estimated prevalence in UK schoolchildren of 1 in 250 3.
Acquired Ichthyosis (AI) is rare and occasionally a paraneoplastic manifestation.
Associated diseases
Malignancy
- non-Hodgkin lymphoma
- Kaposi sarcoma
- multiple myeloma
- leiomyosarcoma
- breast
- ovarian
- cervical
Non-malignancy
- autoimmune
- systemic inflammatory disease
- endocrine/metabolic disorders
- HIV infection
- medication-related
Generalized scaling and dryness of the skin with flaking and itching. On visual inspection is a classic phenotype.
Inherited ichthyosis is divided into syndromic and non-syndromic forms. Mutations have now been found in more than 25 different genes 3.
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Treatment and prognosis
Generally symptomatic.
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1. Takeichi T, Akiyama M. Inherited ichthyosis: Non-syndromic forms. (2016) The Journal of dermatology. 43 (3): 242-51. doi:10.1111/1346-8138.13243 - Pubmed
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2. Oji V, Tadini G, Akiyama M, Blanchet Bardon C, Bodemer C, Bourrat E, Coudiere P, DiGiovanna JJ, Elias P, Fischer J, Fleckman P, Gina M, Harper J, Hashimoto T, Hausser I, Hennies HC, Hohl D, Hovnanian A, Ishida-Yamamoto A, Jacyk WK, Leachman S, Leigh I, Mazereeuw-Hautier J, Milstone L, Morice-Picard F, Paller AS, Richard G, Schmuth M, Shimizu H, Sprecher E, Van Steensel M, Taïeb A, Toro JR, Vabres P, Vahlquist A, Williams M, Traupe H. Revised nomenclature and classification of inherited ichthyoses: results of the First Ichthyosis Consensus Conference in Sorèze 2009. (2010) Journal of the American Academy of Dermatology. 63 (4): 607-41. doi:10.1016/j.jaad.2009.11.020 - Pubmed
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3. Schmuth M, Gruber R, Elias PM, Williams ML. Ichthyosis update: towards a function-driven model of pathogenesis of the disorders of cornification and the role of corneocyte proteins in these disorders. (2007) Advances in dermatology. 23: 231-56. Pubmed
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