Results of a nationwide epidemiologic survey of autosomal recessive congenital ichthyosis and ichthyosis syndromes in Japan.
J Am Acad Dermatol
; 81(5): 1086-1092.e1, 2019 Nov.
Article
em En
| MEDLINE
| ID: mdl-30268591
ABSTRACT
BACKGROUND:
Autosomal recessive congenital ichthyosis (ARCI) and ichthyosis syndrome (IS) are rare genetic skin disorders.OBJECTIVE:
To estimate the number of patients with ARCI and IS in Japan and clarify the clinicoepidemiologic features of these diseases.METHODS:
We performed a nationwide survey of patients treated for ARCI or IS during January 2005-December 2009. We developed diagnostic criteria and conducted a primary survey in a stratified random sample of Japanese hospitals to quantify the number of outpatients and inpatients with ARCI or IS. We performed a secondary survey of clinicoepidemiologic features in positive cases.RESULTS:
The estimated number of patients receiving treatment for ARCI and IS during 2005-2009 was 220 (95% confidence interval [CI] 180-260). The estimated disease distribution was as follows 95 (95% CI 80-110) patients with nonbullous congenital ichthyosiform erythroderma, 30 (95% CI 20-40) with lamellar ichthyosis, 15 (95% CI 10-20) with harlequin ichthyosis, and 85 (95% CI 50-120) with IS.LIMITATIONS:
Patients with a mild case of the disease might not have visited a dermatology department, potentially causing underestimation of affected patients.CONCLUSION:
We report the estimated number of patients with ARCI and IS in Japan and sex differences in the age distribution.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Eritrodermia Ictiosiforme Congênita
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Ictiose
Tipo de estudo:
Diagnostic_studies
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Observational_studies
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Prevalence_studies
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Risk_factors_studies
Limite:
Adolescent
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Adult
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Child
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Female
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Humans
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Male
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Middle aged
País/Região como assunto:
Asia
Idioma:
En
Revista:
J Am Acad Dermatol
Ano de publicação:
2019
Tipo de documento:
Article