Spontaneous corneal perforation in a patient with lamellar ichthyosis and dry eye

Abstract
We report corneal perforation in a patien with lamellar ichthyosis. The patient presented with
complaints of pain, redness, dminished vison, and discharge in her right eye for 15 days. Visual acuities
were light perception in the right and 20/400 in the left eye. Cicatrical ectropion in both lower eyelids
and 2 mm perforation site in the center of the right cornea were observed. Lamellar ichthyosis was
suspected because of scaling and excessive dryness of entire body skin and was confirmed by skin
biopsy. Amnionic membrane transplantasion and transien tarsorraphy was performed and systemic
anti-ichthyiosis therapy was started. The follow-up visits were not possible because of patient
inconsistency. In patients with cicatrical ectropion secondary to ichthyiosis, corneal health should be
closely monitored because of the perforation risk.
Keywords: lamellar ichthyiosis, cornea, spontaneous perforation
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Ocular manifestation of ichthyiosis
Abstract
Purpose: Ichthyiosis is a rare dermato-ocular disease. This study evaluates the oresenting ocular signs,
symptoms, complications, and prognosis of ichthyiosis in a case series from Saudi Arabia.
Methods: A retrospective in chart review was perfoermed for 11 patients with ichthyiosis who
presented to King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia, over thelast 20 years.
Results: The most common presenting ocular diagnosis was ectropion of both the lids. Two patient
developed corneal perforation with poor prognosis. Most of the patients underwent skin grafting to
repair eyelid ectropion. The visual prognosis was excelent because timely surgical intervention was
performed. Hence the ratecorneal complication such as perforation was low.
Conclusion: The most ocular presentation of ichthyiosis is ectropion of both the upper and lower lids.
Despite good visual prognosis, there were some devamstating coneal complications such as
perforation with unpredictable outcomes.
Keywords: Ichthyiosis, Ectropion, Congenital, Skin graft, Collodion
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Eyelid abnormalities in lamellar ichthyoses.
PURPOSE:
To describe eyelid abnormalities in lamellar ichthyoses (LI).
DESIGN:
Retrospective observational case series.
PARTICIPANTS:
Eight patients with classic LI and two patients with congenital ichthyosiform erythroderma.
METHODS:
Results of eyelid and corneal examinations of 10 patients with LI were reviewed and analyzed with
emphasis on the relationship between eyelid ectropion and corneal damage.
RESULTS:
All patients presented with cicatricial lagophthalmos. Of the eight patients with classic LI, five had
ectropion of the four eyelids, one had only lower ectropion, and two had no degree of ectropion.
Two patients with congenital ichthyosiform erythroderma had distinct eyelid abnormalities,
including madarosis and eyelash retraction. Loss of vision caused by corneal damage was found in
three patients with classic LI. Of these three patients, two did not have upper eyelid ectropion.
CONCLUSIONS:
Severe corneal damage can occur in LI even if there is no upper or lower eyelid ectropion.
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Ectropion of all four eyelids associated with severe ichthyosis congenita: a case report
Abstract
Ichthyosis congenita associated with severe ectropion of all four eyelids is an extremely uncommon
condition and has not previously been reported in a Chinese patient.
Our experience of one case shows that a conservative regime of treatment designed to produce a
humidified atmosphere combined with the application of local emulsifying agents can help improve
the severity of the ectropion. Although these effects tend to be temporary this regime allows
surgery to be postponed until the child is older and suitable non-scaly patches of skin can be clearly
identified to serve as skin graft donor sites. In this case, full-thickness grafts from each groin were
successful. Recurrence of the ectropion required a repetition of the skin grafting 18 months later.
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Lamellar ichthyosis presenting as bilateral spontaneous corneal perforation.
Abstract
INTRODUCTION:
Lamellar Ichthyosis is an autosomal recessive, inherited skin disorder characterized by thickening,
fissuring and scaling of the skin.
OBJECTIVE:
To report a case of lamellar icthyosis and highlight the importance of monitoring corneal health in
these patients.
CASE:
We report a rare case of bilateral spontaneous corneal perforation in a patient with lamellar ichthyosis
.The patient presented with complaints of diminution of vision, foreign body sensation, watering and
discharge in both of his eyes for the last three months followed by pain and redness for one week.
Visual acuities were light perception in both the eyes. Cicatricial ectropion was seen in both the lower
eyelids. Corneal perforation was seen in both the eyes. Lamellar ichthyosis was diagnosed on the basis
of scaling and excessive dryness of the entire body skin and was confirmed by skin biopsy.
Peneterating keratoplasty of both eyes was done with cataract surgery of the right. The systemic anti-
ichthyosis therapy was started. Ectropion of the right eye was corrected, and on follow-up at three
months, the patient had a visual acuity of 4 /60 and CFCF in the right and the left eye respectively.
CONCLUSION:
In patients with cicatricial ectropion and dry eye secondary to ichthyosis, corneal health should be
closely monitored because of the perforation risk.
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Spontaneous bilateral corneal perforation in a patient with ichthyosis.
Abstract
We present an 8-month-old female patient with bilateral spontaneous corneal perforation and
lamellar ichthyosis. On examination, the skin of the body and face of the patient was completely dry
and scaly. Cicatricial ectropion and descemetocele with small perforations were seen. Double-layered
amniotic membrane transplantation on the cornea with eyelid construction was performed on both
eyes. At the 6-month follow-up, lower eyelid minimal ectropion formation and inferior corneal
leukoma were seen bilaterally. In ichthyosis patients, the cornea should be monitored closely due to
the risk of severe visual loss and corneal perforation.