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Case Report
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| Epidermolysis bullosa in a small for gestational age preterm male neonate with two affected siblings: A case report | ||||||
| Swathi Chacham1, Jakkampudi Nagasravani2, Uppin Narayan Reddy3, Jillalla Narsing Rao4, S. Pratap Rao5, Kumar AS6 | ||||||
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1MD Pediatrics, DM Neonatology (from Post Graduate Institute of Medical Education and Research, Chandigarh, India), Assistant Professor, Department of Pediatrics, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Andhra Pradesh, India.
2MBBS, (MD, Pediatrics), Junior Resident Doctor, Department of Pediatrics Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Andhra Pradesh, India. 3MD Pediatrics, Professor and Head, Department of Pediatrics Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Andhra Pradesh, India. 4MD Pediatrics, Professor, Department of Pediatrics, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Andhra Pradesh, India. 5MD Pediatrics, Professor, Department of Pediatrics, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Andhra Pradesh, India. 6MD Dermatology (From All India Institute Of Medical Sciences, New Delhi), Professor and Head, Department of Dermatology, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Andhra Pradesh, India. | ||||||
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| Chacham S, Nagasravani J, Reddy UN, Rao JN, Rao SP, Kumar AS. Epidermolysis bullosa in a small for gestational age preterm male neonate with two affected siblings: A case report. Int J Case Rep Images 2014;5(10):691–694. |
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Abstract
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Introduction:
Epidermolysis bullosa is a rare group of hereditary disorders, which are characterized by blistering of the skin and mucosa due to little or no apparent trauma. The severity can range from mild, localized skin blisters to generalized, systemic life-threatening disease and the treatment is mainly supportive. Dystrophic type of epidermolysis bullosa is one of the rarer forms. Skin fragility often leads to secondary skin infections and in turn generalized infection which can be lethal. Hence, the major challenge encountered in the care of a neonate with epidermolysis bullosa is optimum skin care and expert nursing care. A preterm, small for gestational age (SGA) neonate, has immature skin physiologically, which is a portal for systemic infection when compared to a term appropriate for gestational age neonate. The presence of blistering lesions in a preterm, SGA neonate further challenges the management.
Case Report: We report a preterm, low birth weight (small for gestational age) male neonate, who had extensive bullous lesions and dystrophic nails in the first week of life. These lesions healed by scarring leading to stiffness of underlying joints and were complicated by secondary infection. There was history of consanguinity and previous two siblings also had similar lesions with contractures. Conclusion: A preterm male small for gestational age neonate born of consanguineous marriage, with the history of cutaneous bullous lesions in two siblings, presented with extensive bullous lesions and dystrophic nails, suggestive of dystrophic epidermolysis bullosa. | |
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Keywords:
Blistering, Dystrophic, Neonate, Epidermolysis bullosa, Low birth weight, Preterm, Small for gestational age (SGA), Infant
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Author Contributions
Swathi Chacham – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published Jakkampudi NagaSravani – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published Uppin Narayan Reddy – Conception and design, Acquisition of data, Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published Jillalla Narsing Rao – Conception and design, Acquisition of data, Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published S. Pratap Rao – Conception and design, Acquisition of data, Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published Kumar A. S. – Conception and design, Acquisition of data, Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published |
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Guarantor of submission
The corresponding author is the guarantor of submission. |
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Source of support
None |
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Conflict of interest
Authors declare no conflict of interest. |
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Copyright
© 2014 Swathi Chacham et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information. |
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