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Collodion Baby: A Case Report

Article  in  Journal of Perinatology · July 2000

DOI: 10.1038/sj.jp.7200369 · Source: PubMed

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Clinical Perinatal/Neonatal
Case Presentation
䡲 䡲 䡲 䡲 䡲 䡲 䡲 䡲 䡲 䡲
Collodion Baby: A Case Report
Maliha J. Shareef, MD
Phyllis Lawlor-Klean, MS, RNC, CNS
Kathleen A. Kelly, BSN, RNC
Nancy S. LaMear, MD
Michael J. Schied, MD
A case report of a collodion baby born in a community hospital who was
diagnosed, stabilized, and transferred for dermatologic management is
presented. Differential diagnosis based on cornification disorder
phenotypes is outlined. The initial stabilization, management, and
nursing considerations of the infant with impaired barrier function of
the skin are outlined.
Journal of Perinatology 2000; 4:267–269.
The term “ichthyosis” refers to a heterogeneous group of disorders
characterized by abnormal keratinocyte differentiation leading to
excessive scaling of the skin. The severity of scaling can range
from dry skin in mild cases to “collodion membrane,” which is
found in lamellar ichthyosis.
Harlequin ichthyosis, or harlequin fetus, is a severe gener-
alized disorder of epidermal differentiation characterized by
massive generalized hyperkeratosis and usually death in the
perinatal period. Ultrastructurally, harlequin ichthyosis has an
absence of normal lamellar bodies. At birth, the thick stratum
corneum results in constrictive deformation, with severe ectro-
pion and eclabium. Vital functions such as respiration and
swallowing are compromised. Survival in the neonatal period
is rare. Patients with harlequin ichthyosis represent the most
severe end of the phenotypic spectrum of autosomal recessive
ichthyosis (Figure 1).1
Infants born with shiny generalized hyperkeratosis known
as collodion membrane subsequently may develop one of sev-
eral different clinical phenotypes. The severe classic lamellar
ichthyosis phenotype is characterized by large brown thick
scales with a plate-like appearance over the entire body surface
combined with eyelid ectropion. Smaller and lighter scales
characterize the milder phenotype, namely nonbullous con-
genital ichthyosiform erythroderm, with generalized skin red-
Departments of Maternal-Child Services (P. L.-K., K. A. K.) and Obstetrics/Gynecology
(M. J. S.), LaGrange Memorial Hospital, LaGrange, IL; and Pediatrics/Neonatology (M. J. S.,
N. S. L.), Children’s Memorial Hospital, Chicago, IL.
Address correspondence and reprint requests to Maliha J. Shareef, MD, Loyola University
Medical Center, 107–5822, 2160 South First Avenue, Maywood, IL 60153.
Journal of Perinatology 2000; 4:267–269
© 2000 Nature America Inc. All rights reserved. 0743– 8346/00 $15
www.nature.com/jp
䡲 䡲 䡲 䡲
ness. Histologically, lamellar ichthyosis is characterized by mas-
sive orthohyperkeratosis, whereas congenital ichthyosiform
erythroderm may occasionally exhibit areas of parakeratosis in
thickened corneum. Differentiation between the two phenotypes in
the postnatal period is difficult until later in childhood.2,3
Infants born encased in a collodion membrane, later shed,
have episodes of sepsis as well as fluid and electrolyte imbalances
due to impaired skin barrier function. Current treatments are
largely palliative.
CASE REPORT
An appropriate-for-gestational-age infant boy was born at 39
weeks’ gestation to a 30-year-old white female, gravida 1, para 0,
with an unremarkable pregnancy. Elective cesarean section was
performed for breech presentation. Apgars were 9 and 9 at 1 and 5
minutes, respectively. Collodion phenotype was noted at birth. The
physical examination was unremarkable except for ectropion of
eyelids; low-set, small malformed ears; and slight eclabium (Fig-
ure 2). All extremities were edematous and cyanotic with re-
stricted movements. The infant was placed in a humidified iso-
lette after initial stabilization in a radiant warmer. A 10%
concentration of dextrose water was started at a rate of 80 ml/kg
per day. The complete blood count was unremarkable. Prophylac-
tic antibiotics consisted of ampicillin and gentamicin. The baby
was then transferred for further management.
At the tertiary hospital, the baby was placed in an isolette with
humidity. Consultations with dermatology, with otolaryngology to
evaluate the patency of the auricular canal and tympanic mem-
brane, and with ophthalmology consult for corneal abrasions
were obtained. Aquaphor (petrolatum, mineral oil, mineral wax,
and wool wax alcohol; Beiersdorf, Norwalk, CT) was applied twice
daily to the entire body surface area, and lacrilube was applied to
the eyes. Coban was used to secure lines instead of adhesive tapes,
to prevent of skin trauma. The infant was started on oral feedings
on day 2 of life. Electrolytes drawn every eight hours remained
within normal limits. Antibiotics were stopped after 48 hours, as
the blood culture remained negative. The infant was then weaned
gradually from the isolette and was able to maintain his body
temperature. The infant was then discharged from the hospital on
day 5 of life after skin improvement. The parents were instructed
to give short baths with water only once a day and to apply Aqua-
phor twice daily. Follow-up in dermatology, ophthalmology, and
otolaryngology clinics as an outpatient was recommended. At the
267
 Shareef et al. Case Study of a Collodion Baby

Table 1 Stabilization Care of the Collodion Baby

Impaired skin integrity12
Goal: Prevent further impairment
of skin barrier function and maintain
skin integrity to prevent sepsis.
1. Assess open skin lesions.
2. Minimal handling, vital signs
per routine and PRN.
3. Use standard precautions with
handing.

Risk for altered body temperature 1. Avoid radiant heat.

Goal: Minimize TEWL and heat loss
while maintaining skin temperature
between 36.5°C and 37°C.
2. Isolette, with high humidity
(may need to use respiratory
high-humidity hood if
nonhumidified isolette).
3. Pre-warm all direct skin
contact linen used to absorb
weeping.

Altered nutrition: Less than body 1. Evaluate enteral feeding

requirements ability, preferred
Goal: Provide the most appropriate 2. Closely monitor hydration:
nutritional management based on the I&O, moist mucous
infant’s presentation/tolerance. membranes, weight,
evaporimeter measurement,
etc.

Sleep pattern disturbance 1. Position for comfort with

Goal: Promote normal infant sleep/ flexed positioning or by
wake states. holding.

them.4,5 Proper thermoregulation is established by placing the infant

Figure 1. Harlequin fetus. Reprinted with permission of Neonatal Network.
2-month follow-up visit, the baby had normal skin with a few
areas of mild dry skin (Figure 3).
DISCUSSION
Due to the impaired barrier function of the skin, caregivers must
recognize the possibility for potential problems and help to minimize
Figure 2. Collodion baby in case study.
268
in a heated isolette with a minimum of 40 to 60% humidity.6 An um-
bilical vein catheter placed for hydration, using minimal tape and a
pectin barrier to maintain the integrity of the skin should be used.
Parental bonding is encouraged by allowing the parents to be at the
bedside as much as possible during the stabilization period. Touching
and holding the infant need not be forbidden. Table 1 outlines the
individualized plan of care for this infant.5,7,8
Skin impairment gives rise to transepidermal water loss (TEWL),
percutaneous infection, and toxicity. Accurate intake and output is
essential, as is the accurate monitoring of electrolytes for hypernatre-
mia and the appropriate adjustments of fluid intake. Measurement of
TEWL with an evaporimeter should be considered.
After the initial stabilization and convalescent period, the caregiv-
ers must anticipate the discharge needs of the infant and family. This
includes anticipatory guidance in skin care. Skin care concerns and
management are similar to those for a preterm infant.
The appearance of a collodion baby is described in most pediatric and
neonatology texts; however, the incidence is very low. Identification of this
condition and immediate and proper care of the infant are the chief priori-
ties. Morbidity and mortality in these children during the neonatal period
is increased, as the result of an impaired skin barrier function. The im-
paired barrier places the infant at risk for increased water loss, thermal
instability, percutaneous toxicity, and infection.
In a term collodion baby, the infant is at risk for TEWL and the
associated heat loss that accompanies evaporation. Buyse et al.9 found
that the TEWL values in a collodion baby were initially very high and
that improvement during the first month of life paralleled the clinical
improvement of the skin. TEWL decreases exponentially as the gesta-
Journal of Perinatology 2000; 4:267–269
Case Study of a Collodion Baby
Figure 3. Case study infant at his 2-month follow-up visit.
tional age of the infant increases. Water loss is 10 to 15 times greater
in preterm infants born at 25 weeks’ gestation. TEWL is measured in
grams per square meter of body surface per hour. It is measured using
an evaporimeter to determine the water vapor pressure gradient above
the skin surface, which is in direct proportion to the rate of water loss
from the skin. Nopper et al.10 evaluated the use of a water-in-oil emol-
lient cream in preterm infants between 30 and 36 weeks’ estimated
gestational age. Twice daily use of Aquaphor decreased the TEWL by
80% in the first 30 minutes and by 37% in the next 4 to 6 hours.10 We
extrapolated this information to the care of this infant with abnormal
skin development who was at increased risk for TEWL. We used Aqua-
phor to minimize TEWL of the skin. The electrolytes were within nor-
mal range, indicating that the TEWL was minimal, which we viewed
as proof of the efficiency of Aquaphor to decrease TEWL.
Collodion babies are at high risk for infection due to the impaired
barrier function of the skin. In the study by Nopper et al.,10 the Aqua-
phor-treated groups also had a 3.3% rate of sepsis versus 26% in the
control group. Our infant was treated with Aquaphor, and close atten-
tion was given to hand-washing. Subsequently, no episodes of infec-
tion developed in the hospital or at home.
Application of topical products carries a risk of percutaneous
intoxication. Extensive application of 10% urea cream to two collo-
dion babies resulted in a raised plasma urea concentration, which
Journal of Perinatology 2000; 4:267–269
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Shareef et al.
returned normal after the preparation was no longer used.11 Heat
regulation may be problematic in collodion babies, and hyperpyrexia
may occur if the ambient temperature of the environment is too high.
Application of the water-in-oil emollient cream did not significantly
increase the cutaneous temperature.10 As both lamellar ichthyosis and
congenital ichthyosiform erythroderm have been reported to be inher-
ited as autosomal recessive trait, genetic counseling was offered to the
parents but was declined due to lack of family history.
In conclusion, we have reviewed the existing literature concern-
ing collodion baby syndrome and have described a case that appears
to represent a mild form of congenital nonbullous ichthyosiform
erythroderma. The skin treatment with Aquaphor was used to mini-
mize TEWL and its subsequent risk of infection. In our experience
through this case study, we recommend careful attention to skin care,
minimal use of skin care products, and meticulous attention to pre-
venting infection when caring for an infant with collodion baby syn-
drome.
Acknowledgments
We thank the Maternal Child Services staff at LaGrange Memorial Hospital and the
Children’s Memorial Hospital transport team and neonatal intensive care unit staff.
Photographic credit is extended to Paul M. Martinez, BA, of the Audiovisual Department
at LaGrange Memorial Hospital. Assistance with manuscript preparation is extended to
Tina Dolgner, Chris Todd, BGS, RN, and Daniel Polk, MD.
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