Ocular changes in a case of Kyrle's disease. 20-year follow-up.
OBJECTIVE
To re-evaluate a case of Kyrle's disease with conjunctival and corneal changes in a 52-year-old patient with onset at the age of 5 years.


METHODS
Repeated biopsies from the skin, conjunctiva and oral mucosa along with a corneal button obtained from a right eye keratoplasty were examined histopathologically.


RESULTS
The skin, oral, conjunctival and corneal histopathology showed parakeratosis, dyskeratotic cells and single cell keratinization. The clinical picture and the histopathological findings are consistent with the diagnosis of Kyrle's disease.


CONCLUSIONS
This is the first reported case of Kyrle's disease with conjunctival changes. We suggest that Kyrle's disease, although predominantly affecting young adults, may also be seen in childhood. The disease may be a genodermatosis. It is not confined only to the skin, but can be seen in mucous membranes like the conjunctiva and buccal mucosa, and in tissues having the same embryological origin as the skin, i.e. the cornea.
Alyahya
G A
GA
Eye Pathology Institute, University of Copenhagen, Denmark.
Heegaard
S
S
Prause
J U
JU
D017668:Age of Onset; D003228:Conjunctiva / Q000473:pathology*; D003229:Conjunctival Diseases / Q000175:diagnosis*; D003315:Cornea / Q000473:pathology*; D003316:Corneal Diseases / Q000175:diagnosis*; D007644:Darier Disease / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006801:Humans; D015948:Keratoplasty, Penetrating; D008297:Male; D008875:Middle Aged; D009061:Mouth Mucosa / Q000473:pathology; D012867:Skin / Q000473:pathology