Kenza Khachani, Meryeme Marhraoui, Mariame Meziane, Laila Benzekri, Nadia Ismaili
Journal: Medpeer Publisher
ISSN: 3066-2737
Volume: 3
Issue: 9
Date of Publication: 2026/09/06
Darier's disease is a rare autosomal dominant genodermatosis with a chronic, relapsing course that can pose diagnostic challenges during severe flares. We report the case of a 30-year-old woman with a longstanding history of Darier's disease presenting with an extensive, erosive flare involving the neck, trunk and flexural folds. Dermoscopy revealed a pinkish background with characteristic polygonal, star-like brown areas, while onychoscopy showed a pathognomonic V-shaped notch with red-and-white longitudinal bands. Histology confirmed suprabasal acantholytic dyskeratosis with typical round bodies and grains. Notably, dermoscopy also uncovered post-inflammatory hypopigmentation, a feature not typically seen in other inflammatory dermatoses. This case highlights dermoscopy and onychoscopy as fast, accessible, non-invasive tools that sharpen clinical suspicion, guide biopsy site selection and support long-term monitoring of Darier's disease, offering real potential to shorten the diagnostic pathway for this rare but manageable disorder.
Darier's disease, genodermatosis, dermoscopy, polygonal brown areas, onychoscopy, V-shaped notch
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