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Papular Acantholytic Dyskeratosis of the Vulva: A Case Report and Literature Review.
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 PDFDarier disease: Current insights and challenges in pathogenesis and management.
J Eur Acad Dermatol Venereol
November 2024
Department of Dermatology and Venereology, Kepler University Hospital Linz, Linz, Austria.
Darier disease is a rare autosomal dominant genodermatosis caused by mutations in the ATP2A2 gene encoding for sarcoendoplasmic reticulum Ca ATPase isoform 2. The skin disease is characterized by a chronic relapsing course with recurrent reddish-brown keratotic papules and plaques located mainly in seborrhoeic areas. Due to chronic inflammation and epidermal barrier defects of the skin, patients often develop severe bacterial and viral superinfections.
View Article and Find Full Text PDFSegmental Darier's Disease Treated With Cryotherapy.
Cureus
October 2024
Dermatology, Lehigh Valley Health Network, Allentown, USA.
Darier's disease is characterized by pruritic keratotic papules resulting from dysfunctional keratinocyte adhesion. Segmental Darier's is a rare variant thought to be caused by a post-zygotic somatic mutation. The mainstay of treatment consists of oral and topical retinoids and corticosteroids.
View Article and Find Full Text PDFA novel ATP2A2 mutation in Darier and genotype phenotype: correlation analysis.
Genes Genomics
January 2025
North China University of Science and Technology Affiliated Hospital, Tangshan, 063000, Hebei, China.
Background: Darier's disease (DD) is a skin disorder caused by mutations in the ATP2A2 gene. Researchers have been investigating the correlation between genotype and phenotype in DD. Understanding the genotype-phenotype relationship in DD can enhance our comprehension of the genetic background and phenotypic characteristics of the condition, as well as the relationship between its systemic and localized manifestations.
View Article and Find Full Text PDFAcantholytic Dyskeratoses of the Vulva: Clinicopathologic Characterization of 16 Cases and Review of the Literature.
Int J Gynecol Pathol
August 2024
Department of Pathology, Michigan Medicine, University of Michigan, Ann Arbor, Michigan.
Article Synopsis
- The study focuses on vulvar acantholytic dyskeratoses, which are rare skin conditions affecting the vulva and perineum, with a goal to improve classification and understanding for gynecologic pathologists.
- Between 1990 and 2023, 16 cases of vulvar acantholytic dyskeratoses were analyzed, mostly affecting women aged 36 to 79, with many presenting asymptomatic or pruritic lesions predominantly on the labia majora.
- Histologically, these conditions showed features like acantholysis, with some patients responding to local excision or corticosteroids; however, no clear distinction was found between sporadic and syndromic cases based on
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