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CASE kEFOkI

A 1 day premature baby girl was hospitalized in


neonatal care unit 3 weeks ago with a membrane
covering the whole body, presented in an hours after
birth. On the examination there were yellowish
membrane on the whole body and in several areas of
the body the membrane started to peeling off on the
second day. From the ophthalmology examinations
we found bilateral ectropion, which was worse in the
left eye and conjungtivitis on the both eyes. The rest
anterior segmen were normal. There was also
F|gure 2. chtyosis in te whole body
eversion of the lips found in this patient. There were
no other congenital anomalies. The baby was born
premature (35-36 weeks of gestation) and low birth
weight (2000 grams). No history of consanguinity
from the parents. The dermatologist diagnosis was
lamellar ichthyosis. The baby was treated in neonatal
care unit, with humidifid environment and given
intravenous antibiotics (penicillin) for 5 days, Natrium
Fusidat cream and wet dressing for the skin,
Polymisin-neomysin eyedrops 4 times daily for 5
days and Vitamin Apalmitat eye gel 3 times daily. On
the sixth day the membrane on the eyelids were
peeling off and the ectropion were disappeared. The
skin was also smoother. The patient was discharged
on the eleventh day with advised to continue the
emollient and regular check up for the condition of the
eye and skin. F|gure 3. The ecteropion and Eclabium was
disappeared after 6 days
treatment
F|gure 4. The condition was looked well when the F|gure1. Bilateral Ectropion and Eclabium
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entire body and lasts for days to weeks. Till INIkODbCIION
1968 a total of 103 cases had been reported in Collodion baby often a manifestation of lamellar
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world literature. ncidence of this condition is 1 in ichthyosis. Lamellar ichthyosis is a rare type of
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ichthyosis manifesting at birth often encased in 300,000 live births. Bilateral ectropion is the
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collodion membrane and large thick scales all over main ocular involvement in lamellar ichthyosis. t
the body, except mucous membrane and lips. is important to attempt early treatment to prevent
Collodion baby is a rare congenital disorder further complications due to corneal exposure.
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resembling harlequin foetus but milder in degree. We report a case of l amel l ar i chthyosi s
Collodion babies are born with a taut, shiny, r epr esent Col l odi on baby wi t h bi l at er al
translucent or opaque membrane that encases the ectropion in first day of life.
A8SIkACI
Furpc:e: To report a case of lamellar ichthyosis represent Collodion baby with bilateral ectropion in first day of life.
MeIhcc: A case report, 1 day old premature baby girl hospitalized with a membrane covering the whole body,
presented in an hours after birth, and started breaking in the second days. On examination the whole body was
covered with membrane resembling collodion and was peeling off on the chest, abdomen and legs. Associated
ocular findings included bilateral ectropion, which worse in the left eye and conjunctivitis. There were no other
congenital anomalies. Routine blood and urine analysis were within normal limits. Baby was treated in humidified
environment with prophylactic antibiotics. Emollient was applied to the whole body, and the eyelids were treated
with topical antibiotics and lubricant. Fe:u|I: The baby recovered well. On the sixth day the membrane in her
eyelids were peeling off and the ectropion were disappeared. The baby was discharged with advice for regular
emollient application and check up. Ccnc|u:icn: Collodion baby represents difficult treatment challenge because

of prematurity, dehydration, temperature instability, and infection. Therefore supportive care is important to
prevent mortality.
Keywcrc:: ectropion, lamellar ichthyosis, baby
Ccrre:pcncence: qbal Hilmi, c/o: Departemen/SMF lmu Kesehatan Mata Fakultas Kedokteran Universitas
Airlangga/RSU Dr. Soetomo. Jl. Mayjend. Prof. Dr. Moestopo 6-8 Surabaya 60286. E-
mail: iqbalhilmi07@yahoo.co.id
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1urnal Oftalmologi Indonesia
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BILATERALECTROPION IN COLLODION BABY
Iqbo| H||m|, Ar|esont| Ir| Hondoyon|, kotno Doem||oh
Department of Ophthalmology, Airlangga University, Dr. Soetomo Hospital, Surabaya
I S S N . 1 6 9 3 - 2 5 8 7 Jurnal Oftalmologi ndonesia Vol. 6, No. 2, Agustus 2008 : Hal. 134 - 136 135 Jurnal Oftalmologi ndonesia Vol. 6, No. 2, Agustus 2008
Bilateral Ectropion
emollients. The point to be remembered is to avoid DISCbSSION
urea containing agents because of chances of Lamellar ichthyosis is one of the rare congenital
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developing high plasma urea levels. Other drugs that ichthyosiform dermatoses . chthyosis belongs to the
are toxic like salicylates and retinoids should be group of genodermatoses, characterized by

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avoided. There is no need to operate immediately for
hyperkeratosis and desquamation of the epidermis.
ectropion as it can be corrected by local application of
The word ichthyosis; is derived from a Greek work "
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clobetasol in older children. ikthus " meaning fish. chthyosiform dermatosis, are
a group of hereditary desorders characterised by
CONCLbSION dryness and roughness of the skin with excessive
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Collodion baby represents difficult treatment accumulation of epidermal scales. Congenital
challenge, because of prematurity, dehydration, ichthyoses which affect the entire body in the
temperature instability, and infection. Therefore perinatal period include several major subtypes;
supportive care is most important to prevent mortality. harlequin ichthyosis, lamellar ichthyosis (L), and
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congenital ichthyosiform erythroderma.
Collodion babies occur uncommonly although
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not as rarely as the harlequin baby. The
REFERENCES
characteristic feature of the disease is a thin, dry,
1. Dhaded SM, Havaldar PV, Siddibhavi BM, Patil
shining, brownish-yellow parchment-like membrane
VD. Collodion baby. ndian Journal of
which completely envelopes the newborn. This gives
Dermatology, Venereology and Leprocy, 1992,
a collodion or "backed apple" look to the newborn and
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vol 58(6),p393-394
such children are called "collodion babies".
2. Bale,SJ. Autosomal recessive congenital
Ocular manifestations of ichthyosis vary
2
ichtyosis. Gene Review. 2007.
according to the type of ichthyosis. Scales on
3. Lone A, Untoo RA, Ahmad SS. Bilateral Severe
eyelashes and eyelids may be seen in all varieties.
Ecteropion and mature cataract in Lamellar
However, the tight collodion membrane covering the
ichtyosis. JK Sciences, 2004, vol 6 no3,p157-
newborn and producing ectropion of lids is
3 158.
characteristically found in lamellar ichthyosis.
4. Boparai MS, Dash RG, Sahi BK. Lamellar
Clinical manifestation is heterogeneous and
ichthyosis (collodian baby) with severe bilateral
depends on the type of the disease. chthyosis is a
ectropion. ndian Journal of Ophthalmology,
skin disorder characterized by excessive dryness of
3 1988, vol 36(3),p140-141.
skin and increased formation of epidermal scales.
5. Cakmak A, Baba F, Cakmak, Shermatov K,
At the terminal differentiation of keratinocytes, a
Karazeybek H. Treatment Of Congenital
variety of proteins specific for keratinization are
chthyosis With Acitretin.
expressed and unique subcellular structures are
formed. Some of these structures and proteins are
thought to be closely associated with the
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pathogenesis of severe congenital ichthyosis.
Prenatal diagnosis is now possible, using
sonography, which shows ectropion, eclabium, nasal
or aural abnormalities, and hypoplasia of the
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extremities.
Collodion baby represents difficult treatment
challenge, because of prematurity, dehydration,

temperature instability, and infection. Therefore
supportive care is most important to prevent
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mortality.
Skin can be made soft and moist by applying
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136 Jurnal Oftalmologi ndonesia Vol. 6, No. 2, Agustus 2008
Bilateral Ectropion