|
 |
| CASE REPORT |
|
| Year : 1988 | Volume
: 36
| Issue : 3 | Page : 140-141 |
|
Lamellar ichthyosis (collodian baby) with severe bilateral ectropion
MS Boparai, RG Dash, BK Sohi
Commandant, Base Hospital, Delhi Cantt - 110 010, India
Correspondence Address:
M S Boparai
Commandant, Base Hospital, Delhi Cantt - 110 010
India
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 3255705 
A case of lamellar ichthyosis (collodian baby), is being reported. Skin biopsy has confirmed the diagnosis. Severe bilateral ectropion of thee eyelids was the prominent feature. Management of such cases has been briefly discussed.
How to cite this article:
Boparai M S, Dash R G, Sohi B K. Lamellar ichthyosis (collodian baby) with severe bilateral ectropion. Indian J Ophthalmol 1988;36:140-1 |
| Introduction | |  |
The word ichthyosis; is derived from a Greek work 'ikthus' meaning fish. Ichthyosiform dermatosis, are a group of hereditary desorders characterised by dryness and roughness of the skin with excessive accumulation of epidermal scales. Lamellar ichthyosis, is one of the rare congenital ichthyosiform dermatoses [1]. The characteristic feature of the disease is a thin, dry, shining, brownish-yellow parchment-like membrane which completely envelopes the newborn. This gives a collodion or "backed apple" look to the newborn and such children are called "collodion babies". This condition was first described by Seelingman in 1841. Ballantyne was the first author to make an extensive report on 33 cases. Till 1968 a total of 103 cases had been reported in world literature [2].
We are reporting a case of lamellar ichthyosis, with bilateral ectropion of the eye lids in addition to the generalised involvement of the body surface by a membrane.
| Case Report | | |
A 27 years old lady delivered a full term female baby weighing 2.7 kg on 13 Sep. 1977 after uncomplicated labour. The baby was covered all over with a brown coloured, dry, shining membrane. Due to tautness of the membrane the baby's eyes were remaining open with marked ectropion of the lids of both eyes and the mouth was fixed in an open position [Figure - 1]. The baby was not able to suck or cry. While being observed the membrane cracked at neck and groins within a few hours of birth.. Treatment was initiated with frequent light massage of the entire body surface with white soft paraffin. Methyl cellulose was instilled to each eye frequently to prevent exposure keratitis. A wet saline gauze was lightly placed over the face covering the eyes totally. Feeding was carried out through nasogastric tube. About 2 days after birth the baby could move her mouth sufficiently and was able to suck and cry. She was able to close the eyes adequately after two days and ectropion totally disappeared in a week's time.
The massage with white soft paraffin was continued and after about three months the membrane disappeared from the body surface except over the forehead where a number of big dry scales persisted [Figure - 2]. A skin biopsy done from the left groin about 24 hours after birth showed thickening of the stratum corneum and stratum granulosum alongwith perivascular lymphocytic infiltration and the orifices of hair-follicles by keratice precipitating [Figure - 3]. History revealed a nonconsanguinous marriage of the parents with their earlier two children having died soon after the birth. In the last delivery a female child with a similar membrane over her body was born, but died one hour after birth.
| Discussion | | |
Lamellar ichthyosis is known to be an inherited autosomal recessive disorder [3] Though not a feature in our case, 8% of such cases give a history of consingunity. Similarly, our case was a female child though the disease is reported to be two times more common in males. 25% of such children born premature and 51% have similarly affected siblings. Our case was born at' full term but had a similarly affected sister who died soon after birth. Although 80% patients show a generalised involvement, others have the desease limited either to the trunk or one or two extremities. Flexor aspects of the body are most severely affected. The other reported associations of the disease are bilateral ectropion (33%), dimished or absent sweating (10%), nail dystrophies (less than 5%) and seasonal recurrence of the dermatosis in summer (15%). These children are extra susceptible to systemic infection [4] .Alternate formation and shedding of scales on the skin from time to time [5] has been described. The histopathology has been discussed and marked hyperkeratosis, normal to thickned granular layer on occasion prominent rete ridges was found [6].
Follicular orifices are filled with keratin and hair follicles and sebaceous glands are smaller than normal. There is universal agreement regarding management 3 of such cases with plain ointments, keratolytic preparations and in severe cases administration of systemic and topical corticosteroids.
The present case illustrates the severity of ectropion of eyelids and high-lights the importance of proper treatment to prevent exposure keratopathy.
| References | | |
| 1. | Duke Elder. S., System of Ophthalmology Vol. XIII, Henry Kimpton, London, p.p. .257-259, 1968.  |
| 2. | Lentz, C.L. and Attman, J., Arch. Derm., 97:3-13, 1968. |
| 3. | Wells, R.S, B.M.J., 2:1504-1506, 1966. |
| 4. | Bloom, D. and Goodfried, M.S., Arch. Derm., 86:336-342, 1962. |
| 5. | Wells, R.S and Kerr, C.B., Arch Derm., 92:1-5, 1965. |
| 6. | Frost, P., and Scott. E.J.V., Arch. Dcrm., 94:113-126, 1966. |
[Figure - 1], [Figure - 2], [Figure - 3]
|
.png "Santen")
Search Pubmed for