التفاصيل البيبلوغرافية
| العنوان: |
Clinico-Epidemiologic Profile of Non-Syndromic Congenital Ichthyosis -- A Retrospective Chart Review of 107 Patients. |
| المؤلفون: |
Mahajan, Rahul, Bakshi, Shirin, Chatterjee, Debajyoti, De, Dipankar, Saikia, Uma N., Handa, Sanjeev |
| المصدر: |
Indian Journal of Dermatology; Mar/Apr2024, Vol. 69 Issue 2, p113-118, 6p |
| مصطلحات موضوعية: |
DISEASE duration, SKIN diseases, RETROSPECTIVE studies, TERTIARY care, DESCRIPTIVE statistics, CHI-squared test, AGE factors in disease, CONGENITAL ichthyosiform erythroderma, MEDICAL records, ACQUISITION of data, ICHTHYOSIS, X-linked genetic disorders, DATA analysis software, VASCULAR diseases, VITAMIN D, SYMPTOMS |
| مصطلحات جغرافية: |
INDIA |
| مستخلص: |
Background: Congenital ichthyoses are a rare Mendelian group of disorders affecting the integument with a heterogeneous clinical presentation amongst which scaling is a constant feature. There is scanty epidemiologic data regarding the clinical profile and histologic patterns of inherited ichthyosis from resource-poor countries. Aims and Objectives: The study was aimed at assessing the clinic-epidemiologic characteristics associated with the different forms of non-syndromic congenital ichthyosis. Materials and Methods: This was a retrospective chart review of ichthyosis patients that presented between July 2016 and Jun 2020. Details including demographic profile, clinical characteristics along with any relevant investigations done were included. Results: During the study period of 4 years, 107 patients with congenital non-syndromic ichthyosis were seen. The most frequent diagnosis was of common ichthyoses, followed by autosomal recessive congenital ichthyosis, epidermolytic ichthyosis and erythrokeratoderma, in decreasing order. Conclusion: Important clinical findings like erythema and the type of scales as well as histological differences including an absent or reduced granular layer in ichthyosis vulgaris can help differentiate among the clinical phenotypes of inherited non-syndromic ichthyosis especially in resource-poor settings. Also, there is a high prevalence of vitamin D deficiency and hence a need for screening for the same in all patients of congenital ichthyosis including the milder phenotypes. [ABSTRACT FROM AUTHOR] |
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| قاعدة البيانات: |
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