2024-03-28T13:24:14Z
https://nagoya.repo.nii.ac.jp/oai
oai:nagoya.repo.nii.ac.jp:00027957
2023-01-16T04:43:44Z
499:500:501
Cross-sectional survey on disease severity in Japanese patients with harlequin ichthyosis/ichthyosis: Syndromic forms and quality-of-life analysis in a subgroup
Murase, Chiaki
91029
Takeichi, Takuya
91030
Shibata, Akitaka
91031
Nakatochi, Masahiro
91032
Kinoshita, Fumie
91033
Kubo, Akiharu
91034
Nakajima, Kimiko
91035
Ishii, Norito
91036
Amano, Hiroo
91037
Masuda, Koji
91038
Kawakami, Hiroshi
91039
Kanekura, Takuro
91040
Washio, Ken
91041
Asano, Masayuki
91042
Teramura, Kazuya
91043
Akasaka, Eijiro
91044
Tohyama, Mikiko
91045
Hatano, Yutaka
91046
Ochiai, Toyoko
91047
Moriwaki, Shinichi
91048
Sato, Tomotaka
91049
Ishida-Yamamoto, Akemi
91050
Seishima, Mariko
91051
Kurosawa, Michiko
91052
Ikeda, Shigaku
91053
Akiyama, Masashi
91054
Clinical ichthyosis score
Harlequin ichthyosis
Congenital ichthyosis
Syndromic form ichthyosis
Quality of life
Background: Congenital ichthyoses (CIs) adversely affect quality of life (QOL) in patients. However, the effects of CIs on patient QOL have not been studied sufficiently. Objective: To investigate the association between disease severity and QOL in patients with harlequin ichthyosis (HI) and ichthyosis: syndromic forms (ISFs) Methods: Clinical information of patients with HI and ISFs from 2010 to 2015 were obtained from 100 dermatology departments/divisions of principal institutes/hospitals throughout Japan. We examined the relationship between disease severity and QOL in patients with HI and ISFs. Patients who were aged 8 years or older and participated in a multicenter retrospective questionnaire survey in Japan were assessed by dermatology life quality index (DLQI, range of 0–30) and clinical ichthyosis score (range of 0–100). Results: Netherton syndrome patients had a significantly higher risk of allergy to food or environmental allergens than patients with other phenotypes. Keratitis-ichthyosis-deafness (KID) syndrome patients showed a significantly higher risk of skin infections than patients with other phenotypes. Complete data on DLQI were obtained from 13 patients, whose median age was 21 (8–71) years. Nine patients were male, and 4 were female. Systemic retinoids were administrated to 2 of the 3 HI patients. The Spearman’s correlation coefficient between the clinical ichthyosis score and DLQI was 0.611 (P < 0.05). Conclusion: We confirmed that Netherton syndrome and KID syndrome patients have a higher risk of allergy to food or environmental allergens and of skin infections, respectively. QOL impairment correlates with disease severity in HI and ISFs patients.
ファイル公開:2019-11-01
journal article
Elsevier
2018-11
application/pdf
Journal of Dermatological Science
2
92
127
133
0923-1811
https://nagoya.repo.nii.ac.jp/record/27957/files/Revise.pdf
eng
© 2018. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/