TY - JOUR
T1 - Corneal abnormalities in incontinentia pigmenti
T2 - Histopathological and confocal correlations
AU - Selvadurai, Deepan
AU - Salomão, Diva R.
AU - Baratz, Keith H.
N1 - Copyright:
Copyright 2021 Elsevier B.V., All rights reserved.
PY - 2008/8
Y1 - 2008/8
N2 - 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 ̃7-20 μm 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, therewas 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.
AB - 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 ̃7-20 μm 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, therewas 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.
KW - Bloch-Sulzberger syndrome
KW - Confocal microscopy
KW - Incontinentia pigmenti
KW - Transmission electron microscopy
KW - Whorllike corneal epitheliopathy
UR - http://www.scopus.com/inward/record.url?scp=49849106772&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=49849106772&partnerID=8YFLogxK
U2 - 10.1097/ICO.0b013e31816b6a26
DO - 10.1097/ICO.0b013e31816b6a26
M3 - Article
C2 - 18650672
AN - SCOPUS:49849106772
SN - 0277-3740
VL - 27
SP - 833
EP - 836
JO - Cornea
JF - Cornea
IS - 7
ER -