A rare case of bullous congenital ichthyosiform erythroderma in a newborn
Keywords:
Bullous Congenital ichthyosiform erythroderma, Keratinization disorder
Abstract
Bullous congenital ichthyosiform erythroderma is a rare disorder of keratinization. It is associated with defective keratinization leading to skin fragility, blistering and hyperkeratosis. This condition was difficult to distinguish from staphylococcal scalded skin syndrome. We report a case of Bullous congenital ichthyosiform erythroderma in a 72 hour old male baby and discussed the differential diagnosis and management of the disease.
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References
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2. DiGiovanna JJ, Bale SJ. Clinical heterogeneity in epidermolytic hyperkeratosis. Arch Dermatol. 1994 Aug;130(8):1026-35. [PubMed]
3. Cheng J, Syder AJ, Yu QC, Letai A, Paller AS, Fuchs E. The genetic basis of epidermolytic hyperkeratosis: a disorder of differentiation-specific epidermal keratin genes. Cell. 1992;vol 70:[ 811-819]. [PubMed]
4. Chipev CC1, Korge BP, Markova N, Bale SJ, DiGiovanna JJ, Compton JG, Steinert PM. A leucine----proline mutation in the H1 subdomain of keratin 1 causes epidermolytic hyperkeratosis. Cell. 1992 Sep 4;70(5):821-8. [PubMed]
5. Kwak J, Maverakis E. Epidermolytic hyperkeratosis. Dermatol Online J. 2006 Sep 8;12(5):6. [PubMed]
6. El Shabrawi-Caelen L, McCalmont TH. Perinuclear eosinophilic bands: a clue to keratin gene mutation. J Cutan Pathol. 2010 Jul;37(7):718-9. doi: 10.1111/j.1600-0560.2010.01555.x. [PubMed]
7. Bergman R, Khamaysi Z, Sprecher E. A unique pattern of dyskeratosis characterizes epidermolytic hyperkeratosis and epidermolytic palmoplantar keratoderma. Am J Dermatopathol. 2008;vol 30: pg[101-105].
8. Kempers S, Katz HI, Wildnauer R, Green B. An evaluation of the effect of an alpha hydroxy acid-blen skin cream in the cosmetic improvement of symptoms of moderate to severe xerosis, epidermolytic hyperkeratosis, and ichthyosis. Cutis. 1998; vol 61:pg [347-350].
CITATION
DOI: 10.17511/ijpr.2016.i01.15
Published: 2016-01-31
How to Cite
Dr Thumjaa Annamalai, Dr Vamsikrishna Reddy kandriga, & Dr Sindhu maru. (2016). A rare case of bullous congenital ichthyosiform erythroderma in a newborn. Pediatric Review: International Journal of Pediatric Research, 3(1), 77-79. https://doi.org/10.17511/ijpr.2016.i01.15
Section
Case Report
Copyright (c) 2016 Author (s). Published by Siddharth Health Research and Social Welfare Society
This work is licensed under a Creative Commons Attribution 4.0 International License.
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