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New disease category of skin disorders proposed

Autoinflammatory keratinization diseases (AIKD), a new class of skin disorders, with a genetic cause rooted in the body's innate immune system © Nagoya University

A team of researchers from Nagoya University in Japan have defined a new type of inflammatory keratinization disorder with autoinflammatory pathogenic mechanisms, which they suggest will improve diagnosis and treatment of rare skin conditions.

Their recent article, published in the Journal of Allergy and Clinical Immunology (June 28, 2017), advocated for the novel and unique concept of autoinflammatory keratinization diseases (AIKDs). They propose the following definition of AIKD. First, the primary and main inflammation sites are the epidermis and the upper dermis. Second, inflammation in the epidermis and upper dermis leads to hyperkeratosis, which is the main and characteristic phenotype of AIKDs.

“Many skin disorders are put into the very broad group of inflammatory keratinization diseases. This term is not very helpful because it covers so many kinds of skin problems and doesn’t consider if the root cause is inflammatory or genetic, or a combination of both,” said Dr. Masashi Akiyama, lead author of the study who is with the Department of Dermatology at Nagoya University, in a press release.

In the experiments conducted, CARD14 mutations in keratinocytes were thought to be responsible for the disease pathology and inflammation of keratinization diseases. AIKD describes inflammatory keratinization disorders with auto-inflammatory mechanisms that include minor cases of psoriasis and other related diseases such as Pityriasis rubra pilaris ( PRP type V) and Keratosis lichenoides chronica (KLC ).

“We have been finding more and more of these auto-inflammatory related skin diseases and it’s time we recognize this as a new category of inflammatory keratinization disease,” said co-author Dr. Kazumitsu Sugiura.

“A better understanding of the root causes of skin problems is the only way for physicians to help patients manage their conditions and develop more effective treatments.”

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