Clinical and pathologic eyelid alterations in a patient with Darier disease

Darier disease (DD) is a rare autosomal dominant dermatosis that has infrequent ocular manifestations, especially those involving the eyelids. The authors describe a patient with long-standing DD who presented with both classic and unique clinical findings. Eyelid biopsy samples studied with electron microscopy demonstrated histopathological changes consistent with DD. The authors postulate how clinical findings not previously reported as "classic" to DD may be associated. To the authors' knowledge, electron micrographs detailing changes associated with DD have not been published for eyelid tissue.