One of the relatively rare forms of the congenital ichthyosiform dermatoses is the collodion baby. ' In this condition, the body of the newborn is encapsulated in whole or in part by a parchment-like membrane which begins to slough, shortly after birth. Bilateral ectropion of the upper lids is a relatively common ophthalmic finding in this rare entity.1 We report the case of collodion baby accompanied by severe congenital bilateral upper lid ectropion with spontaneous resolution.
Report of a Case
A two-hour-old Caucasian female was first seen in consultation at the Childrens Hospital Medical Center because of severe bilateral ectropion. The child was the product of a normal pregnancy and uncomplicated spontaneous vaginal delivery to a gravida 2, para 2, Rh(+) mother. The child weighed 3100 grams at birth with a head circumference of 33 cm and a length of 44 cm. The Apgar score was 4 to 5 with poor coloration.
On physical examination the child was grossly edematous; a parchment-like membrane covered the entire body like a cocoon. Multiple fissures, especially at the joint creases, had a "scaled skin" appearance. The chest was clear to auscultation and no cardiac abnormalities were found.
Severe bilateral upper lid ectropion and ectlabium (Figure 1) were present. The lids were edematous and partially covered by a scaled membrane which was desquamating in some areas. Adequate examination of the corneas was not possible at this time.
The initial electrocardiogram and chest x-ray were normal. The hemogram showed a leukocytosis of 29,000 and a hematocrit of 58 grams percent. The child was given intravenous antibiotics and the skin was treated topically with a 10% urea solution.
Two days later the ectropion diminished as the cutaneous membrane sloughed. At this time, the anterior segment was visualized and was unremarkable. By the sixth hospital day the ectropion had almost completely resolved; the corneas remained clear and spontaneous blinking was present. During sleep, full lid closure and an intact Bell's reflex was observed.
Two months later, there was no evidence of ectropion; the lids were normal except for fine bran-like scales (Figure 2). Slit-lamp biomicroscopy of the corneas and other members of the family was normal.
Ichthyosis is a descriptive term for a group of dermatological disorders characterized by clinically evident deciduous cutaneous scales. Wells and Keer,: in 1965, were able to classify the majority of patients with ichthyosis into four categories based upon clinical inheritance patterns. More recently, the ocular manifestations of 62 patients with ichthyosis were reviewed by Jay and associates1 and they were able to find a definite relationship between the various ocular abnormalities and the genetic classifications. The collodion baby is probably a phenotypic expression of one of several different genotypes, including the nonbullous congenital ichtyosiform erythroderma (an autosomal recessive pattern) or as sex-linked ichthyosis (an x-linked condition).'
FIGURE I: Severe bilateral extropion and ectlabia on admission. Desquamation of parchment-like membrane is also evident in some areas (arrow).
The original description of the collodion baby was reported by Seeligman4 in 1841. This rare congenital ichthyosiform dermatoses is characterized by the appearance of a membrane resembling parchment which completely envelops the child at birth. After birth, fissures develop, particularly in the areas of the large joints, with eventual desquamation of the membrane.
Lentz and Altman' reported on the natural clinical course of this disorder and found that approximately 33% of patients had bilateral ectropion. Spontaneous resolution is common and treatment should be conservative; tarsorrhapy should be delayed as long as possible." The cornea should be protected with ointments if lagophthalmos exists. In our case the ectropion appeared to be beginning to resolve within several days, and was essentially complete within one week. Followup examination two months after the patient's discharge from the hospital revealed only a slight scaling of the skin around the eyelids.
Recent reports have indicated an enzymatic defect may be associated with the X-linked form of ichthyosis.6,8 Roller and Wells determined the enzymatic levels of steroid sulphatase in cultured fibroblasts from 25 patients with X-linked ichthyosis and found markedly reduced levels of this enzyme when compared to controls or other patients with other forms of ichthyosis. The diagnosis of this condition through the use of amniocentesis is a future possibility. Sever and associates* examined a large group of patients with various forms of ichthyosis and found deep stromal opacities of the cornea associated with all of the patients with X-linked ichthyosis and most of the female carriers. Slit-lamp biomicroscopy of the patient and the patient's family failed to reveal any significant corneal abnormalities.
FIGURE 2: Complete resolution of ectropion two months later. Residual scaling of skin is apparent.
In conclusion, when ophthalmologists encounter this rare disorder conservative management of ectropion is indicated and slit lamp biomicroscopic examination of the anterior segment may be important for genetic counseling purposes.
We described a case of severe bilateral ectropion occurring in a collodion baby which spontaneously resolved over a one-week period. No other ocular abnormalities were found on follow-up examination.
1. Orth DH, Fretzin DF. Abramson V: Collodion baby with transient bilateral upper lid ectropion. Arch Ophthalmol 91:206-207, 1974.
2. Wells RS, Kerr CB: Genetic classification of ichthyosis. Arch Derm 92:1-6, 1965.
3. Jay B, Black RK, Wells RS: Ocular manifestations of ichthyosis. Brit J Ophthalmol 52:217-226, 1968.
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