Korean J Dermatol.
2025 Jan;63(1):1-4.
A Case of Type 1 Segmental Darier’s Disease with Bilateral Presentation
- Affiliations
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- 1Department of Dermatology, Pusan National University School of Medicine, Busan, Korea
- 2Department of Dermatology, Pusan National University Hospital, Busan, Korea
- 3Department of Dermatology, Pusan National University Yangsan Hospital, Yangsan, Korea
- 4Biomedical Research Institute, Pusan National University Hospital, Busan, Korea
Abstract
- Darier’s disease is characterized by greasy and scaly papules that primarily affect seborrheic and intertriginous areas which is caused by a mutation in the ATP2A2 gene. Histopathologically, the disease is characterized by acantholysis and dyskeratosis. Among the diverse presentations, the segmental type follows a linear distribution along the lines of Blaschko. Herein, we present a case of a 54-year-old male with generalized erythematous papules that had been linearly distributed across his body for two decades. Lesions on his trunk and extremities were confined to the right side, whereas those on the scalp and face exhibited multiple segmental presentations. Histopathological examination revealed acantholysis and dyskeratosis in the epidermis, confirming the diagnosis of type 1 segmental Darier’s disease. This case underscores the rarity of type 1 segmental Darier’s disease, particularly with multiple segmental involvement and highlights the complexity and variability of this dermatological condition.
