Autoinflammatory Keratinization Diseases View Full Text


Ontology type: schema:Chapter      Open Access: True


Chapter Info

DATE

2021-09-02

AUTHORS

Takuya Takeichi , Masashi Akiyama

ABSTRACT

Inflammation caused by the hyperactivation of innate immunity due to genetic factors occasionally leads to inflammatory cutaneous keratinization diseases. Such inflammatory keratinization diseases with genetic autoinflammatory mechanisms are called “autoinflammatory keratinization diseases” (AiKDs). We proposed a four-part definition of AiKDs. (i) The sites of primary and main inflammation are the epidermis and the upper dermis. (ii) The inflammation at these sites leads to hyperkeratosis, which is the main and characteristic phenotype of AiKDs. (iii) The primary genetic causative factors are associated with the hyperactivation of innate immunity (autoinflammation), mainly in the epidermis and upper dermis. (iv) The concept encompasses diseases with mixed pathomechanisms of autoinflammation and autoimmunity.Several diseases have been categorized as AiKDs: pustular psoriasis and related disorders, pityriasis rubra pilaris, keratosis lichenoides chronica, hidradenitis suppurativa, porokeratosis, and keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK) syndrome. The recognition of innate causative/predisposing issues and the exact assessment of their roles in disease etiology from the perspective of AiKDs promise to bring innovations that will provide more accurate, targeted, causal treatments for various AiKDs. More... »

PAGES

3-20

Book

TITLE

New and Emerging Entities in Dermatology and Dermatopathology

ISBN

978-3-030-80026-0
978-3-030-80027-7

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/978-3-030-80027-7_1

DOI

http://dx.doi.org/10.1007/978-3-030-80027-7_1

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1140807373


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