alexa Colloidion Baby: A Treatment Challenge for Dermatologist and Neonatologist | OMICS International
ISSN: 2167-0897
Journal of Neonatal Biology
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Colloidion Baby: A Treatment Challenge for Dermatologist and Neonatologist

Adeesh Jain1, Deepak Sharma2* and Aakash Pandita3
1Department of Pediatrics, PGIMS, Rohtak, Haryana, India
2Maheshwari Hospital, Bhatinda, Punjab, India
3Department of Pediatrics, GMC, Jammu, India
Corresponding Author : Deepak Sharma
Maheshwari Hospital, Bhatinda, Punjab, India
Tel: +919462270002
E-mail: [email protected]
Received October 15, 2014; Accepted October 17, 2014; Published October 20, 2014
Citation: Jain A, Sharma D, Pandita A (2014) Colloidion Baby: A Treatment Challenge for Dermatologist and Neonatologist. J Neonatal Biol 3:i101. doi:10.4172/2167-0897.1000I.101
Copyright: © 2014 Jain A et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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Case

A term male newborn with birth weight of 3.4 kg with normal Apgar score of 8/9/9 was referred within hours of birth because of presence of parchment like skin all over the body. The detailed examination showed broad thick scales all over the body with predominance over chest, abdomen and upper limbs. There was associated eversion of eyelids and lips. There were excess of scales around face giving appearance of fish like mouth (Figures 1 and 2). There was no other associated anamoly. The infant was managed with adequate humidification with fluid and electrolytes management. Topical application of emollients was done as applied as advised by dermatologist. The scales resolved over next three weeks and baby was discharged. The genetic analysis showed mutation in TGM1 Gene located on chromosome 14q12 which confirmed the diagnosis of Lamellar ichthyosis.

Discussion

Lamellar ichthyosis (ichthyosis lammellaris/nonbullous congenital ichthyosis), is a rare autosomal recessive inherited skin disorder, with incidence of 1 in 600,000. The affected newborns are born in a collodion membrane which is a shiny waxy outer layer to the skin [1]. This membrane is shed 10–14 days after birth with exposure of underlying skin having extensive scaling because of hyperkeratosis. These newborns are usually premature and small for gestational age. As the skin is parchment like at birth, hence they have distortion of facial features with ectropion, eclabium and false contracture of fingers. The biopsy of the skin is nonspecific with and shows compact and thickened stratum corneum [2]. There are many conditions associated with collodion baby and include [3]

• Lamellar Icthyosis

• Non bullous ichthyosiserythroderma

• X-linked ichthyosis

• Netherton syndrome

• Gaucher disease

• Trichothiodystrophy (Tay syndrome)

• Sjogren-Larsen syndrome

• Lamellar exfoliation of the newborn

Careful history taking include family history, evolution of skin condition and associated malformations should be sought. The Lamellar Ichthyosis (LI) is most commonly associated with collodion baby. The most common type LI1 involves mutation of TGM1 gene on chr14q12 [4]. Treatment involves proper humidification, fluid and electrolyte balance, strict asepsis, bland occlusive ointment emollient application, prevention of overheating, eye care and genetic counselling of the parents for further pregnancy depending upon the diagnosis. Gene therapy is also paving this way in the treatment and may become an important tool in future [5]. The eyes must be protected by using artificial tears, band occlusive dressing or surgical intervention. Manual removal of the skin is not recommended. Usually the thickened stratum corneum get resolves within 2 to 4 weeks [6].

Author’s Contribution

Dr Deepak Sharma and Dr Aakash made the primary manuscript, took the photographs and helped in writing manuscript, Dr Adeesh Jain did final corrections of manuscript before submission.

References


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