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| CLINICAL ARTICLE |
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| Year : 2021 | Volume
: 22
| Issue : 2 | Page : 127-130 |
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Collodion baby syndrome: A case study
Joy Priyadharishini1, F Lydia Evelyn2
1 Assistant Professor, College of Nursing CMC, Vellore, Tamil Nadu, India
2 Clinical Instructor, Scudder Memorial Hospital, Ranipet, Tamil Nadu, India
| Date of Submission | 07-Jul-2020 |
| Date of Decision | 01-Feb-2021 |
| Date of Acceptance | 05-Feb-2021 |
| Date of Web Publication | 31-Jan-2022 |
Correspondence Address:
Mrs. Joy Priyadharishini
College of Nursing, CMC, Vellore, Tamil Nadu
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/IJCN.IJCN_64_20
Collodion baby syndrome is a rare congenital autosomal recessive type of ichthyosis. It is a skin disorder characterised by parchment-like taut membrane which is present during birth. Collodion syndrome responds best to medical and nursing measures and may improve or resolve with the treatment of underlying condition. The nurse needs to provide emotional support to the family and impart knowledge to the care provider regarding the treatment choices and resources available. The disease process, common investigations and nursing management of collodion baby are explicated in this article. A case history is briefed with the specific nursing care provided by the community health nurse at the primary healthcare setting.
Keywords: Collodion baby, congenital disorder, ichthyosis, lamellar ichthyosis
How to cite this article:
Priyadharishini J, Evelyn F L. Collodion baby syndrome: A case study. Indian J Cont Nsg Edn 2021;22:127-30 |
| Introduction | |  |
Change the line as follows The term collodion baby was designed by Hallopeau et al.[1] Collodion baby refers to newborn who appears to have parchment-like waxy shiny and taut collodion membrane which sheds within 2 weeks of time.[2] According to Chung et al., only 270 cases have been reported from 1892 to 2015, and according to the Foundation for Ichthyosis and Related Skin Types, 1 in 100,000 cases occur,[3] amongst which 10% of the collodion baby syndromes are self-healing with limited intervention.[4]
| Aetiology | | |
Incidence is equal in both male and female babies, and it affects all populations. It is diagnosed at birth and continues throughout life and may resolve with adequate treatment, follow-up and management.[5]
| Pathophysiology | | |
Collodion baby syndrome is mainly due to the disordered cornification in the squamous epithelial cells, where stratum corneum cells fail to separate, which is due to the mutation of common gene TGM1 (transglutaminase in chromosome 14912). Other genes are also likely to be affected which are CYP4F22, ABCA12, ALOX12B, ALOXE3, NIPAL4 and PNPLA1. Due to disordered cornification in the epithelial cells, the physiological functions such as desquamation, hydration regulation, immune response, activity of dendritic cells and permeability to remove irritants and toxins fail.[6]
| Classification | | |
Collodion baby syndrome comes under the classification of ichthyosis which is classified on the basis of biochemical, histological and molecular genetic criteria by William and Elias in 1985. The most common type of ichthyosis which is presenting as collodion during birth is divided into two major types.[1] They are
- Non-bullous congenital ichthyosiform erythroderma (NBCIE)
- Lamellar ichthyosis (LI).
The common clinical feature which differentiates between NBCIE and LI is according to the scaling and intensity of the erythroderma.[7],[8] The differences are given in [Table 1]. | Table 1: Difference between non-bullous congenital ichthyosiform erythroderma and lamellar ichthyosis forms of collodion baby syndrome
Click here to view |
Neither ultrastructural nor histopathological features will clearly distinguish LI and NBCIE. Mutations of any of the genes which is mentioned in pathogenesis can lead to NBCIE or LI type of collodion syndrome.
Clinical Manifestations
The Clinical Manifestations are outlined in [Table 2]
| Diagnosis | | |
The diagnosis is done based on the clinical manifestation at the time of birth. Genetic testing can reveal the mutation of the specific gene responsible for the disorder. Skin punch biopsy is done to reveal the state of the stratum corneum, targeted gene sequence in the infant's blood.[8],[9]
| Prognosis | | |
Life span of collodion babies depends upon the severity and the intensity of the disease. Most of the babies die within few days of life due to complications such as dehydration, inability to feed, respiratory difficulty and septicaemia.[3] Self-healing collodion baby's get healed within the first few weeks of life.[4]
| Management | | |
Collodion baby syndrome can be treated according to the severity with appropriate medical and nursing management.
The major focus of management is supportive and includes:
- Providing humidified environment by placing the baby in an incubator
- Providing intravenous fluid to prevent dehydration
- Administering prophylactic antibiotics to prevent sepsis
- Applying topical application like bland emollients or petroleum jelly (e.g., Vaseline gauze) for the skin
- Administering eye drops or ointment (e.g., tobramycin) for eye infections
- Topical steroids to treat skin abrasions
- Instilling artificial tears if there is severe ectropion
- Nasogastric tube feeding to address feeding issues
- Administering acitretin (a second-generation retinoid) and nitrofurazone solution bath is shown to be highly effective in treatment of lamellar ichthyosis.[1],[2],[6],[10],[11]
| Complications | | |
Neonatal complications occur in 45% of all collodion babies which lead to mortality rate of 11% during the first few weeks of life.[1],[10] The common complication encountered are:
- Septicaemia
- Electrolyte imbalance
- Renal and neurological damage
- Respiratory distress
- Pneumonia
- Myocardial injury.
| Nursing Management | | |
Community nurses play a vital role in caring for and educating the care provider of a child with Collodion baby syndrome. Timely intervention, appropriate health education and follow-up care will ensure good prognosis and effective coping amongst the care providers.
| Case Report | | |
A term female neonate was delivered by suction cup due to non-reassuring foetal status in a tertiary hospital. The APGAR score was 9 and 9 at 1 and 5 min, respectively, weighing 2550 g at birth. On examination, the vitals of the newborn were normal. A detailed physical examination showed generalised oedema over the body and parchment-like membrane covering the body, and the baby also presented with ectropion and mild eclabium. No other abnormalities were found. The family history revealed that their first child died after 8 days of birth due to collodion baby syndrome. Her antenatal period was uneventful.
The newborn was admitted in neonatal intensive care unit and was nursed in an incubator with 90% humidity. Moisol eye drops were instilled every fourth hourly, and sterile liquid paraffin was applied for the body every 6 h. The baby was treated with intravenous fluid therapy and antibiotics. Initially, the baby was on nasogastric tube feeds. When the baby started sucking, the child was started on direct breastfeeding. The baby was discharged after 10 days of close monitoring. The child was reviewed on the 10th day at the time of discharge and was noted to be shedding the membrane.
The child is 6 years old now and has no signs and symptoms of complications except the dry skin for which she applies liquid paraffin. Regular home care services are rendered by the community health nurse. Health education regarding skin care management is taught to the child and the family members. The caregivers have been taught for the early identification of the complications and to seek medical attention immediately.
Nursing care involves assessment, early identification of symptoms, complications and providing appropriate intervention and health education.[12] Nursing care for a newborn with collodion baby syndrome is discussed using nursing process approach.
1. Nursing Diagnosis
Risk for impaired skin integrity related to improper cornification of the skin.
Expected outcome
Improved skin integrity as evidenced by shedding of skin sheath, wound healing and the baby's skin remains intact and free of irritation and breakdown.
Intervention
- Assessed the skin for erythema and desquamation on every visit and the child was identified to have skin peeling on and off in the past 6 years. Reassured the mother and the child during every visit
- Advised the mother on the importance of providing bath regularly with soap and water
- Advised the mother on the importance of moisturising the skin with emollients such as liquid paraffin
- Provided information on the importance of avoiding irritating soaps or other powders and exposure to triggers such as local injury to skin and intense sun exposure.
Evaluation
The child's skin integrity was preserved.
2. Nursing Diagnosis
Disturbed body image related to change in the physical appearance, secondary to changes in the skin integrity.
Expected outcome
The child will be able to accept and demonstrate enhanced self-esteem and accept bodily changes.
Intervention
- Assessed the social participation and interpersonal relationships and the child's behaviour towards the physical appearance
- Evaluated the child's verbal remarks about the change
- Taught the child and the caregiver on the nature of the disease
- Motivated the caregiver and the child to involve in activities of daily living and help the child identify positive behaviours
- Supported the child in identifying ways of coping.
Evaluation
Perception of the body image was enhanced and the child was able to accept the disease and its progress. The child is now active and cheerful and goes to school.
3. Nursing Diagnosis
Risk for infection related to impaired skin integrity and presence of dead skin.
Expected outcome
The child remains free of infection as evidenced by normal vital signs and absence of signs and symptoms of infection.
Intervention
- Monitored vital signs on every visit to identify any fever and tachycardia
- Taught the caregiver about proper handwashing technique before handling the child
- Demonstrated the mother or the caregiver to apply the prescribed sterile liquid paraffin and Moisol eye drops in a sterile manner
- Advised the mother on the importance of prevention of infection techniques.
Evaluation
Infection was prevented and the child remained healthy.
4. Nursing Diagnosis
Risk for pain related to inflammation of the skin.
Expected outcome
The child will remain without pain and remain comfortable as evidenced by verbalisation.
Intervention:
- Assessed the child for pain during every visit
- Asked the child about any factors that alleviate pain
- Advised the child and mother on importance maintaining the skin integrity
- Advised the mother to report any pain and inflammation to the healthcare providers.
Evaluation
The child remained pain free throughout 6 years.
5. Nursing Diagnosis
Risk for imbalanced nutrition related to difficulty in swallowing related to eclabium.
Expected outcome
The child will be able to eat and remain healthy as evidenced by normal weight gain and ability to attain milestones.
Intervention
- Assessed the weight and appearance of the child. The child looked healthy and had normal growth and development
- Advised the child and mother on importance of well-balanced diet
- Advised the mother to report any difficulty in feeding the child to the healthcare providers immediately.
Evaluation
The child remained healthy and active throughout the past 6 years. The child weighs 17 kg now at 6 years of age.
| Conclusion | | |
Collodion baby syndrome is a rare genetic disorder characterised by ichthyosis. The disease process has a varied severity from being mild non-threatening disease to having severe life-threatening issues. Medical management is mainly supportive. Nursing care involves assessment, early identification of symptoms, complications and providing appropriate intervention and health education.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Chung M, Pittenger J, Tobin S, Chung A, Desai N. Expedient treatment of a collodion baby. Case Rep Dermatol Med 2011;2011:803782.  |
| 2. | Das R, Das JK, Deshmukh S, Shrivastava R, Gupta K. Bilateral ectropion in collodion baby – Three case reports. Int J Ocul Oncol Oculoplasty 2018;4:61-3. |
| 3. | Simalti AK, Sethi H. Collodion baby. Med J Armed Forces India 2017;73:197-9. |
| 4. | Raghunath M, Hennies HC, Ahvazi B, Vogel M, Reis A, Steinert PM, et al. Self-healing collodion baby: A dynamic phenotype explained by a particular transglutaminase-1 mutation. J Invest Dermatol 2003;120:224-8. |
| 5. | Kaur S, Kaur G, Rawat HC, Sethi A. Collodion baby: A case report. Int J Contemp Pediatr 2018;5:2017. |
| 6. | Choudhary R, Sachdeva G, Katoch G, Kumar R. Collodion baby – Congenital ichthyosis: Clinical review. Int J Contemp Paediatr 2019;6:2746-9. |
| 7. | Akiyama M, Sawamura D, Shimizu H. The clinical spectrum of nonbullous congenital ichthyosiform erythroderma and lamellar ichthyosis. Clin Exp Dermatol 2003;28:235-40. |
| 8. | Richard G. Autosomal recessive congenital ichthyosis. In: Gene Reviews. [Internet]. 2001. [cited 2020 Jun 20]. Available from: https://www.ncbi.nlm.nih. [Last accessed on 2020 Jun 20]. |
| 9. | National Institutes of Health. Lamellar ichthyosis. In: Genetics Home Reference. [Internet]. 2015. [cited 2020 Jun 20]. Available from: http://ghr. nlm.nih.gov/condition/lamellar ichthyosis. |
| 10. | |
| 11. | |
| 12. | Gulanick M, Myers JL. Nursing Care Plans: Diagnoses, Interventions, and Outcomes. Philadelphia: Elsevier Health Sciences; 2011. |
[Table 1], [Table 2]
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