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he term collodion baby (CB), first introduced in 1884,1 describes the phenotype of a
newborn with a parchmentlike membrane
covering its body surface.2 Although most patients
will ultimately be given a diagnosis of lamellar
ichthyosis or congenital ichthyosiform erythroderma, a range of outcomes with varying clinical
severity is seen. In the neonatal period, management
is focused on minimizing transepidermal water loss
(TEWL), supporting caloric needs, monitoring and
treating complications, and performing diagnostic
studies. This review summarizes recent advances in
the understanding of CB with emphasis on a practical
approach to management.
CLINICAL CHARACTERISTICS
CB presents at birth with erythroderma and shiny,
tight skin resembling parchment (the so-called collodion membrane) covering the entire body (Fig 1).
In rare instances, a localized collodion membrane
occurs.3 The tautness of the skin may distort features
From the Departments of Dermatologya and Pediatrics,b University of Colorado School of Medicine, Aurora; and Dermatology,
Kaiser Permanente, Denver.c
Funding sources: None.
Conflicts of interest: None declared.
Reprint requests: Renata Prado, MD, Department of Dermatology,
University of Colorado School of Medicine, 1665 Aurora Ct, Mail
Stop F703, Aurora, CO 80045-2517. E-mail: renata.prado@
ucdenver.edu.
Published online August 2, 2012.
0190-9622/$36.00
2012 by the American Academy of Dermatology, Inc.
http://dx.doi.org/10.1016/j.jaad.2012.05.036
1362
Abbreviations used:
CB:
EM:
HI:
NICU:
NS:
TEWL:
TGM:
collodion baby
electron microscopy
harlequin ichthyosis
neonatal intensive care unit
Netherton syndrome
transepidermal water loss
transglutaminase
ASSOCIATED DIAGNOSES
It is important to remember that CB is an initial
presentation of several genetic conditions, and the
eventual phenotype depends on the underlying
J AM ACAD DERMATOL
Prado et al 1363
1364 Prado et al
J AM ACAD DERMATOL
DECEMBER 2012
sepsis,5 and percutaneous toxicity from topical medications.33 Pneumonia secondary to aspiration of
squamous debris in the amniotic fluid has also been
observed.4,6 Mortality in CB was as high as 50% in
1960, decreased to 11% in 1986, and today is about
5%.2,20 The overall prognosis has improved thanks to
advances in neonatal care and the development of
neonatal intensive care units (NICU).6
Practical recommendations regarding the management of CB (Table IV) are as follows:
NEONATAL MANAGEMENT OF CB
The potential complications of CB are listed in
Table III. Of utmost concern is the epidermal
barrier, which is equivalent to that of a very
premature baby. The major consequence in the
neonatal period is increased TEWL, up to 7 times
more than normal-appearing skin,30 Buyse et al30
found that TEWL in CB was initially very high, and a
decrease in TEWL during the first month of life
paralleled the skin improvement. Exaggerated
TEWL is associated with heat loss, temperature
instability, and hypernatremic dehydration.4-6 The
heat loss of evaporation also causes significant
caloric drain, which, in association with caloric
expenditures from cutaneous hyperplasia and
chronic inflammation, can be sufficient to account
for the growth failure observed in patients with
moderate to severe barrier abnormalities.31,32
Because of the defective barrier function, these
newborn infants are at increased risk for bacterial
and Candida albicans cutaneous infections,4
Admission to NICU
The CB should ideally be transferred to NICU, or
to an institution with experience in caring for premature babies if NICU is not available.6
Placement in a high-humidity incubator and
close monitoring of body temperature
There is an inverse linear relationship between
ambient relative humidity and TEWL.34,35 Incubators
able to create high ambient humidity decrease TEWL
regardless of epidermal barrier function.34,36
Placement in a humidified incubator soon after birth
prevents hypernatremic dehydration and hypothermia as a result of excessive TEWL,2,4 and is considered the most important measure when treating
these infants.6 Hypohidrosis and overheating can
occur in CB, and incubator temperatures should be
adjusted to avoid overheating these patients.8,37,38
Although the optimal amount of humidification is
not clearly defined, some advocate a minimum of
40% to 60% humidity,33 whereas others suggest 90%
to 100%.6 At our institution, we follow protocols for
premature infants, using a minimum of 60% humidity, with adjustments as needed.
The literature provides no specific criteria for
transitioning the CB from incubator to open crib.39
At our institution, the decision for incubator weaning
is individualized. Typically, the infant is transferred
to an open crib when there is adequate caloric
intake, appropriate weight gain, and absence of
complications associated with the impaired skin
barrier. Transition should not be delayed unnecessarily as, although there is no information on CB, a
study in premature infants showed that delay in
transfer from incubator to open crib may result in
increased time to achieve full oral feedings, decreased growth velocity, and increased length of
hospital stay.40
Monitoring of water and electrolyte balance,
and energy expenditure
Buyse et al30 found that TEWL in a CB at day 4 was
112 g/m2/h, compared with 18 g/m2/h in healthy
babies. Because of increased TEWL, dehydration and
J AM ACAD DERMATOL
Prado et al 1365
Nonsyndromic
ichthyoses
Disease
Lamellar ichthyosis
Congenital
ichthyosiform
erythroderma
Ichthyosis vulgaris
Recessive x-linked
ichthyosis
Epidermolytic
ichthyosis
SHCB
Syndromic
ichthyoses
Pertinent physical
findings37
Ancillary laboratory/
radiologic testing
Collodion membrane
with ectropion and
eclabium, congenital
ichthyosiform
erythroderma,
platelike scaling,
hypohidrosis
Collodion membrane
with ectropion and
eclabium, congenital
ichthyosiform
erythroderma, fine
scaling, hypohidrosis
Xerosis, generalized
scaling sparing
flexural areas, atopic
dermatitis, pruritus,
accentuated
palmoplantar
markings
Generalized scaling
sparing body folds,
neck usually more
severely involved,
corneal clouding,
cryptorchidism
Large erosions, mild
scaling, and
erythroderma at
birth, followed by
hyperkeratosis in first
few months
Collodion membrane at
birth, followed by
brownish scaling
restricted to bathing
suit areas
Nearly complete
resolution of
collodion membrane
and scaling within
first 3 mo
Generalized
ichthyosiform
erythroderma,
hepatosplenomegaly, mild
myopathy, cataract,
short stature, mild
intellectual disability
In situ
transglutaminase-1
assay in cryostat-cut
sections
TGM1, ABCA12,
ALOX12B, NIPAL4
In situ
transglutaminase-1
assay in cryostat-cut
sections
ALOX12B, ALOXE3,
TGM1, ABCA12,
CYP4F22, NIPAL4
FLG
STS
Gene(s)
KRT1, KRT10
TGM1
In situ
transglutaminase-1
assay in cryostat-cut
sections
TGM1, ALOX12B,
ALOXE3
ABHD5
Continued
J AM ACAD DERMATOL
1366 Prado et al
DECEMBER 2012
Table I. Contd
Category
Disease
Conradi-H
unermannHapple syndrome
KID syndrome
Loricrin keratoderma
ARC syndrome
KLICK syndrome
Metabolic
diseases
Holocarboxylase
synthetase deficiency
Pertinent physical
findings37
Ancillary laboratory/
radiologic testing
Generalized
ichthyosiform
erythroderma, brittle
hair, impaired
intelligence, short
stature, with or
without
photosensitivity
Congenital
ichthyosiform
erythroderma in lines
of Blaschko followed
by hyperkeratosis,
follicular
atrophoderma,
stippled epiphyses,
cataracts, asymmetric
facial appearance
Severe generalized or
localized
erythrokeratoderma
with spiky
hyperkeratosis, PPK,
keratitis, hearing loss
Congenital
ichthyosiform
erythroderma, diffuse
PPK with honeycomb
pattern, pseudoainhum, knuckle
pads
Generalized scaling
with sparing of skin
folds, arthrogryposis,
renal tubular
degeneration,
intrahepatic bile duct
hypoplasia with
cholestasis,
metabolic acidosis,
abnormal platelet
function, cerebral
malformation
Congenital ichthyosis,
linear keratosis in
skin folds, sclerosing
PPK
Metabolic acidosis,
hyperammonemia,
organic aciduria,
metabolic
encephalopathy,
poor feeding,
lethargy, respiratory
distress, and
hypotonia
Polarizing microscopy
of hair shaft:
alternating light and
dark bands, other
hair abnormalities,
eg, trichoschisis or
trichorrhexis nodosa
ERCC2/XPD, ERCC3/
XPB, GTF2H5/TTDA,
C7Orf11/TTDN1
(nonphotosensitive)
H&E: calcification in
follicular keratosis in
neonates, bone x-ray
EBP
GJB2 (GJB6)
LOR
VPS33B
EM: hypergranulosis
and abnormally big
keratohyaline
granules
Carboxylases assay in
leukocytes or
cultured skin
fibroblasts
POMP
Gene(s)
HLCS
Continued
J AM ACAD DERMATOL
Prado et al 1367
Table I. Contd
Category
Disease
Others
Hypohidrotic
ectodermal dysplasia
Congenital
hypothyroidism59-62
Pertinent physical
findings37
Ancillary laboratory/
radiologic testing
Congenital
ichthyosiform
erythroderma,
hepatosplenomegaly,
opisthotonus,
hypotonia, facial
anomalies,
arthrogryposis,
respiratory distress,
and progressive
neurologic
deterioration
Hypohidrosis or
anhidrosis with
hyperpyrexia, atopic
dermatitis, sparse
hair, frontal bossing,
saddle nose,
supraorbital ridging,
hypoplastic mid face,
thick everted lips,
hypodontia or
anodontia, pegshaped/conical
incisors and canines,
hypoplastic or absent
breast and nippleareolar complex
Clavicular pad;
puffiness of
periorbital tissues
and tongue, lips,
hands, and genitals;
dry, cold, and pale
skin; dry and brittle
nails and hair; patchy
alopecia; dwarfism;
mental retardation;
somnolence;
constipation; feeding
problems; poor
muscle tone;
persistence of
jaundice; and
respiratory problems
Deficiency of
b-glucosidase in
leukocytes or
cultured skin
fibroblasts, LFTs,
increased serum acid
phosphatase,
Gaucher cells in bone
marrow
GBA
Gene(s)
Continued
J AM ACAD DERMATOL
1368 Prado et al
DECEMBER 2012
Table I. Contd
Category
Disease
Koraxitrachitic
syndrome63
Pertinent physical
findings37
Ancillary laboratory/
radiologic testing
A syndromic form of
SHCB: collodion baby
at birth, with patchy
erythema,
progressive thinning
of hyperkeratotic
membrane,
generalized irregular
dermal atrophy,
alopecia, absent
eyelashes and
eyebrows, and
conjunctival pannus,
hypertelorism,
prominent nasal root,
large mouth,
micrognathia,
brachydactyly,
syndactyly involving
all interdigital spaces,
and camptodactyly
of fingers III-V
Diffuse, nonprogressive
PPK in combination
with intermittently
pruritic, slowly
progressive
anogenital
leukokeratosis
H&E:
orthohyperkeratosis
and marked atrophy
of dermis, immature
dermal extracellular
matrix, factor XIII-a
positive dendrocytes
are rare and globular
rather than dendritic
Unknown
Unknown
Gene(s)
ARC, Arthrogryposis-renal dysfunction-cholestasis; CPK, creatine phosphokinase; EM, electron microscopy; H&E, hematoxylin and eosin; KID,
keratitis-ichthyosis-deafness; KLICK syndrome, keratosis linearis with ichthyosis congenita and sclerosing keratoderma; LFT, liver function test;
MRI, magnetic resonance imaging; NAD, nicotinamide adenine dinucleotide; PPK, palmoplantar keratoderma; SHCB, self-healing collodion
baby; T4, thyroxine; TSH, thyroid-stimulating hormone; TTD, trichothiodystrophy.
J AM ACAD DERMATOL
Prado et al 1369
Harlequin ichthyosis
Ichthyosis prematurity
syndrome
Netherton syndrome
gren-Larsson
Sjo
syndrome
Gene(s)
ABCA12
SLC27A4
SPINK5
ALDH3A2
d
d
d
d
d
d
d
d
d
d
d
d
Admission to NICU
Placement of newborn in highly humidified incubator
Monitoring of body temperature and avoidance of
hypothermia because of increased transepidermal water
loss and overheating caused by hypohidrosis
Monitoring of water and electrolyte balance
Caloric supplementation if necessary
Monitoring for signs of cutaneous or systemic infection,
and standard NICU precautions for infection control
when handling child
Use of petrolatum-based topical emollient several times
a day
Avoidance of medicated ointments
Ophthalmologic evaluation of ectropion
Otorhinolaryngologic evaluation if external ear canal is
obstructed
Pain management if necessary
Nasogastric tube placement if difficulty sucking prevents
adequate feeding
Use of oral retinoid when necessary
Encouragement of parental involvement in care of baby
Diagnosis of underlying condition
(relative risk 1.20, 95% confidence interval 1.001.43) infections compared with standard care.36
There was heterogeneity in the results of the studies
included in this meta-analysis. For example, in the
study by Nopper et al, the water-in-oil emolliente
treated group had a 3.3% rate of sepsis versus 26% in
the control group.41 Taieb and Labreze6 reported
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Prado et al 1371
intermediate filaments in the stratum spinosum. It must be cautioned that the use of EM
for differentiating the ichthyoses depends on
the experience of the user and the methods
used. Elias et al57 have advocated processing
EM specimens using both osmium tetroxide
and ruthenium tetroxide postfixation to visualize lipid-containing structures in addition to
ultrastructural morphology. Although these
studies have been of tremendous use on the
research front, clinical availability is limited.
J AM ACAD DERMATOL
1372 Prado et al
presentation suggestive of Sj
ogreneLarsson syndrome, fatty alcohol oxidoreductase activity can
be measured in fibroblasts, and mutation analysis
to identify fatty aldehyde dehydrogenase gene
mutations can confirm the diagnosis.55,58 Blood
samples may be examined for lipid inclusions in
leukocytes, which are found in neutral lipid storage disease with ichthyosis and Gaucher disease.
DECEMBER 2012
4.
5.
6.
POSTDISCHARGE FOLLOW-UP
We follow up patients every 3 months during the
first year of life in the dermatology clinic after their
discharge from the NICU. More frequent visits may
be necessary in patients with severe disease or who
require treatment with oral acitretin. Genetic counseling should be offered to affected families after an
exact diagnosis has been established.
For management of the ichthyoses after the newborn period readers are encouraged to read an
excellent review published by Oji and Traupe.38
CONCLUSIONS
CB is not a diagnosis but a neonatal phenotype
that can evolve into many distinct conditions, primarily disorders of cornification. The severely compromised epidermal barrier represents the greatest
challenge during the newborn period, and advances
in neonatal care have significantly improved the
prognosis of these babies.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
REFERENCES
1. Hallopeau H, Watelet R. Sur une forme attenuee de la maladie
dite ichthyose foetale. Ann Dermatol Syphiligr 1884;3:149-52.
2. Van Gysel D, Lijnen RL, Moekti SS, de Laat PC, Oranje AP.
Collodion baby: a follow-up study of 17 cases. J Eur Acad
Dermatol Venereol 2002;16:472-5.
3. Mazereeuw-Hautier J, Aufenvenne K, Deraison C, Ahvazi B,
Oji V, Traupe H, et al. Acral self-healing collodion baby: report
20.
21.
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