Annals of Otolaryngology Head and Neck Surgery (ISSN 2835-7132) | Volume 4, Issue 1 | Case Report | Open Access DOI

Rare Otologic Manifestation of Darier's Disease: A Case Report

João Tavares Correia^*

João Tavares Correia, Rita Carvalho, Sofia Carvalho, Mariline Santos, Isabel Silva Carvalho

Unidade Local de Saúde de Santo António - Otorhinolaryngology and Cervical-Facial Surgery Department, Porto, Portugal

^*Correspondence to: João Tavares Correia 

Abstract

This article presents a rare case of pinnae involvement in a 73-year-old female with Darier’s disease, an autosomal dominant genodermatosis characterized by keratotic papules in a seborrheic distribution. The patient, diagnosed at age 16, presented with bilateral aural fullness and hypoacusis, alongside recurrent external ear crusting and debris accumulation, necessitating ongoing management with aural cleaning and topical treatments. Audiometric evaluation confirmed normal hearing thresholds. This case highlights the otologic manifestations of Darier’s disease, emphasizing the need for interdisciplinary care between dermatology and otorhinolaryngology to manage chronic symptoms and maintain patients’ quality of life.

Keywords:

Darier’s disease; Pinnae involvement; External otitis; Keratosis follicularis

Citation:

João Tavares Correia, Rita Carvalho, Sofia Carvalho, Mariline Santos, Isabel Silva Carvalho. Rare Otologic Manifestation of Darier's Disease: A Case Report. Annal of Otol Head and Neck Surg. 2025;4(1):1-3.