Darier's disease presenting with otitis externa

Abstract

Darier's disease is an uncommon genetic disorder that causes dermatological manifestations such as crusted papules at seborrhoeic sites of the head, neck and trunk. Acantholysis and dyskeratosis are the characteristic histopathological picture. Darier's disease is a genetically inherited disease that occurs with haploinsufficiency. Chronic otitis externa along with recurrent dermatological lesions should be investigated to rule out Darier's disease, which will be helpful to provide appropriate treatment. We report the occurrence of Darier's disease manifesting severe otitis externa in a 62-year-old male patient who presented with recurrent bilateral otitis externa with scaly and greasy lesions on the external auditory canal and pinna and was diagnosed to have bilateral otitis externa. At the age of 30 years, he was diagnosed with Darier's disease on the basis of skin biopsy. This recurrent otitis externa with Darier's disease should be known to the clinician for early diagnosis and prompt treatment.

Keywords

• darier's disease
• external auditory canal
• otitis externa
• pinna