Darier disease, previously known as keratosis follicularis, is a rare autosomal dominant genodermatosis characterized by keratotic papules and longitudinal eyrthronychia; it is caused by mutations in the ATP2A2 gene, encoding a calcium pump within the endoplasmic reticulum. Notably, the condition showcases high penetrance but variable expressivity, emphasizing that while each individual inheriting the genotype manifests some form of the disease, its presentation and severity vary across affected persons. Due to its complex expressivity, this activity highlights the importance of recognizing Darier disease, irrespective of a negative familial background. Darier disease is exacerbated by sunlight, heat, infections, and friction. This activity reviews the clinical presentation encompassing multiple red or brown papules with hyperkeratosis, nail anomalies including longitudinal erythronychia, and mucosal changes typically surfacing around puberty and persisting throughout life. By exploring evaluation techniques and treatment modalities, this session empowers healthcare professionals to adopt a holistic approach, focusing on interdisciplinary collaboration to manage this dermatological disorder effectively. The interprofessional team’s role in assessing, diagnosing, and coordinating care for Darier disease patients is underscored, recognizing the impact of external exposures and emphasizing tailored therapeutic strategies to improve patient outcomes and quality of life.
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