Dermatology: Practical and Conceptual Review | Dermatol Pract Concept. 2024;14(2):e2024124 1 Vulvar Acantholytic Warty Dyskeratoma Sabina Vaccari1, Luca Rapparini1,2, Cosimo Misciali1,2, Emi Dika1,2 1 Oncologic Dermatology Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico S. Orsola-Malpighi, Bologna, Italy 2 Department of Medical and Surgical Sciences, Alma Mater Studiorum University of Bologna, Italy Citation: Vaccari S, Rapparini L, Misciali C, Dika E. Vulvar Acantholytic Warty Dyskeratoma. Dermatol Pract Concept. 2024;14(2):e2024124. DOI: https://doi.org/10.5826/dpc.1402a124 Accepted: December 20, 2023; Published: April 2024 Copyright: ©2024 Vaccari et al. This is an open-access article distributed under the terms of the Creative Commons Attribution- NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original authors and source are credited. Funding: None. Competing Interests: None. Authorship: All authors have contributed significantly to this publication. Sabina Vaccari wrote the paper and coordinated the study; Luca Rapparini wrote the paper, collected and processed clinical data, tables and the pictures; Cosimo Misciali processed and analyzed the histological sample; Emi Dika supervised the study. All authors read and approved the final paper. Corresponding Author: Luca Rapparini, Dermatology Unit, Department of Medical and Surgical Sciences, Alma Mater Studiorum, University of Bologna, Bologna, Italy. ORCID 0000-0002-4029-941X Mail: luca.rapparini2@studio.unibo.it Case Presentation A 43-year-old female was examined for an exophytic nodule on the internal side of the small vulvar lip (Figure 1A). She referred the occurrence for 25 years. On clinical examination, the lesion appeared as a well- circumscribed pinkish nodule, with a central umbilicated crater filled with keratin. On dermoscopy, a smooth surface of the lesion, color of the surrounding mucosa, on the pe- riphery is appreciated. In the central area an accumulation of whitish keratotic scales overlying a verrucous- papillary sur- face with linear and corkscrew-shaped vessels was observed (Figure 1B). Condyloma, squamous cell carcinoma, and keratoacan- thoma were hypothesized clinical differential diagnoses, so it was decided to proceed with a radical excision. On histopathologic evaluation, there is well- circumscribed neoplasm with invagination of marked epidermis hyperpla- sia with columns of parakeratosis, dyskeratotic acantholysis containing round keratinocytes separated from one another. Edema, dilated vessels in the superficial chorion (Fig. 1C, D). The histological investigations concluded for acantholytic warty dyskeratoma. Teaching Point Acantholytic warty dyskeratoma is an uncommon benign epidermal proliferation which can involve both the skin and the mucous membranes, in particular the oral mucosa, the pharynx, the perineum, the rectal mucosa, and the vulva [1]. It should be included within the spectrum of dermatoses with focal acantholytic dyskeratosis. Histologically, it is characterized by a crater-shaped epidermal proliferation with prominent dyskeratosis, acantholysis, superimposed by parakeratotic cells, with edema and dilated vessels in the superficial chorion. These histological findings allow the di- agnosis of acantholytic warty dyskeratosis and rule out other common conditions affecting the vulvar region [2]. 2 Review | Dermatol Pract Concept. 2024;14(2):e2024124 References 1. Duray PH, Merino MJ, Axiotis C. Brief Communication Warty Dyskeratoma of the Vulva. Int J Gynecol Pathol. 1983 Mar;2(3): 286–93. 2. Lencastre A, Campos S, Cabete J. Warty dyskeratoma. J Am Acad Dermatol. 2016 Sep;75(3):e97–8. Figure 1. Well-circumscribed pinkish nodule, with a central umbilicated crater filled with keratin (A). A smooth surface of the lesion, color of the surrounding mucosa on the periphery, and an accumulation of whitish keratotic scales overlying a verrucous-papillary surface, with linear and corkscrew-shaped vessels in the central area (B). Well-circumscribed neoplasm with invagination (H&E, 4x) (C) and column of parakeratosis, dyskeratotic acantholysis containing round keratino- cytes separated from one another (H&E, 25x)(D).