当前位置： SCI文献检索 > JOURNAL OF DERMATOLOGICAL SCIENCE期刊下所有文献 > Cross-sectional survey on disease severity in Japanese patients with harlequin ichthyosis/ichthyosis: Syndromic forms and quality-of-life analysis in a subgroup.

Cross-sectional survey on disease severity in Japanese patients with harlequin ichthyosis/ichthyosis: Syndromic forms and quality-of-life analysis in a subgroup.

Abstract：

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.

journal_name

J Dermatol Sci

journal_title

Journal of dermatological science

authors

Murase C,Takeichi T,Shibata A,Nakatochi M,Kinoshita F,Kubo A,Nakajima K,Ishii N,Amano H,Masuda K,Kawakami H,Kanekura T,Washio K,Asano M,Teramura K,Akasaka E,Tohyama M,Hatano Y,Ochiai T,Moriwaki S,Sato T,Ishida-Y

doi

10.1016/j.jdermsci.2018.08.008

subject

Has Abstract

pub_date

2018-11-01 00:00:00

pages

127-133

issue

eissn

0923-1811

issn

1873-569X

pii

S0923-1811(18)30318-9

journal_volume

pub_type

杂志文章

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Cross-sectional survey on disease severity in Japanese patients with harlequin ichthyosis/ichthyosis: Syndromic forms and quality-of-life analysis in a subgroup.