Clinicopathological features of a suspected case of hereditary benign intraepithelial dyskeratosis with bilateral corneas involved: a case report and mini review.

Purpose: To report a suspected case of hereditary benign intraepithelial dyskeratosis (HBID) in China.

Methods: Case report and review of the related literature.

Results: A 43-year-old woman developed bilateral, elevated, white-to-grayish, gelatinous plaques on the perilimbal region and buccal mucosa. Her daughter had similar lesions on the buccal and pharyngeal mucosa. In vivo confocal microscopy demonstrated the loss of limbal palisades of Vogt in the patient's left eye. An incisional biopsy of her conjunctiva and buccal mucosa was performed, which indicated that the histopathological characteristics were quite similar to those of HBID. The patient underwent removal of the plaques and limbal allograft transplantation in the left eye. Postsurgery treatments included systemic cyclosporin A and topical artificial tears for both eyes. Ocular symptoms were alleviated, and no relapse was observed during the 1.5-year follow-up.

Conclusions: In a suspected case of HBID in China, limbal stem cell transplantation was effective.