Darier disease is one of the most common genodermatoses. Although Darier disease was described in 1886, targeted therapies remain unknown. Current literature lacks specific guidelines for treatment of Darier disease. Treatment remains symptomatic and may be challenging for dermatologists. The aim of this article is to present clinical characteristics and treatment options. In a mild form of the disease with a small number of skin lesions, the symptoms can be reduced by the use of topical medications. Oral retinoids, alternatively doxycycline, seem to be beneficial in extensive and persistent lesions unresponsive to local treatment. In limited, hypertrophic forms, surgical methods, laser therapy and photodynamic therapy could be used.
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http://dx.doi.org/10.5114/ada.2022.124344 | DOI Listing |
Clin Cosmet Investig Dermatol
January 2025
Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Papular acantholytic dyskeratosis (PAD) of the vulva is an uncommon benign condition characterized by multiple hyperkeratotic papules in the anogenital region. First described in 1984, PAD belongs to the spectrum of focal acantholytic dyskeratoses and shares histopathological features with Darier disease and Hailey-Hailey disease. Despite its persistence, PAD is benign, requiring only reassurance in many cases.
View Article and Find Full Text PDFJAAD Int
February 2025
Department of Dermatology, Mayo Clinic, Rochester, Minnesota.
Australas J Dermatol
December 2024
Skin Health Institute, Melbourne, Victoria, Australia.
Qual Life Res
December 2024
Department of Health Policy, Corvinus University of Budapest, 8 Fővám Square, Budapest, Hungary, 1093.
Objectives: Limited evidence is available about the content validity of the EQ-5D-5L in rare skin fragility disorders. Previous research has demonstrated that the skin irritation and self-confidence additional dimensions (bolt-ons) improve the content validity of the EQ-5D-5L in psoriasis and atopic dermatitis. Our aim was to investigate the content validity of the EQ-5D-5L and the two bolt-ons in Darier's disease and Hailey-Hailey disease.
View Article and Find Full Text PDFOrphanet J Rare Dis
December 2024
Department of Dermatology and Venereology, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gröna stråket 16, Gothenburg, SE-413 45, Sweden.
Background: Darier disease is a genodermatosis which manifests as hyperkeratotic papules and superficial erosions mainly in seborrheic skin areas. This retrospective registry-based cohort study aimed to estimate the association between Darier disease and skin cancer.
Results: Patients diagnosed with Darier disease were identified from the patient registry of Sahlgrenska University Hospital (Gothenburg, Sweden) in 2016-2020.
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