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Corneal abnormalities in incontinentia pigmenti: histopathological and confocal correlations
Deepan Selvadurai1, Diva R Salomão, Keith H Baratz
1Department of Ophthalmology, Mayo Clinic College of Medicine, Rochester, MN, USA.
Insights
In incontinentia pigmenti (IP), whorllike corneal epitheliopathy presents with epithelial cell degeneration. Treatment focuses on supportive care due to rapid recurrence of this eye condition.
Area of Science:
- Ophthalmology
- Genetics
- Cell Biology
Background:
- Incontinentia pigmenti (IP) is a rare genetic disorder affecting the skin, hair, nails, and central nervous system.
- Ocular manifestations in IP can include various abnormalities, with corneal involvement being less common but significant.
Observation:
- A case of a 22-year-old woman with IP and bilateral whorllike corneal epitheliopathy is presented.
- Confocal microscopy revealed bright, round objects within the corneal epithelium, measuring 7-20 micrometers.
- Histopathology showed epithelial disorganization, degenerating nuclei, and apoptosis.
Findings:
- The corneal pathology in this IP patient was confined to the epithelial layer.
- Surgical debridement provided temporary relief, but the epitheliopathy recurred within one month.
- The observed changes suggest a primary defect in epithelial cell maturation and apoptosis.
Implications:
- Whorllike corneal epitheliopathy in IP appears to stem from abnormal epithelial cell development.
- Supportive treatments like artificial tears and bandage contact lenses are recommended for managing symptoms.
- Further research into the specific cellular mechanisms underlying IP-associated corneal disease is warranted.
Purpose:
To describe the clinical presentation and histopathologic and confocal correlations in a case of incontinentia pigmenti (IP)-associated whorllike corneal epitheliopathy.
Methods:
We describe the case report of a 22-year-old woman with IP and bilateral whorllike corneal epitheliopathy, with symptomatic dry eye complaints. Slit-lamp and confocal microscopic imaging confirmed that the pathology was restricted entirely within the epithelial layer. The cornea was debrided with the hopes of alleviating symptomatic eye complaints. The excised tissue was analyzed by standard light microscopy and transmission electron microscopy.
Results:
In vivo confocal microscopic imaging (ConfoScan 3; Nidek, Fremont, CA) of both eyes showed the presence of multiple bright, round objects approximately 7-20 microm in diameter and located entirely within the epithelial layer. Hematoxylin-eosin-stained sections of the debrided tissue showed a mild disorganization of the overall epithelial architecture. Transmission electron microscopy noted various degrees of degenerating nuclei. The corneal epithelial defect of the patient's left eye healed by the fourth postoperative day. The new epithelium appeared normal, but at the 1-month examination, there was recurrence.
Conclusions:
Histomorphologic evaluation of the surgical specimen showed abnormal changes restricted to the epithelium in our patient. The observed changes are suggestive of a primary abnormality of cell maturation, which results in degeneration, and apoptosis as the epithelial cells migrate upward. The rapid recurrence of keratopathy clearly indicates that supportive treatment of blurred vision and secondary superficial punctate keratopathy with artificial tears, punctal plugs, and bandage contact lens are more likely to be beneficial.
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